1.

Epidemiologi
A.TOF :Congenital cardiopathy is one of the most common problems at birth, with an incidence of 1 to 1.5 in
10,000 live births. Currently, more than 85% of children with congenital cardiopathy survive to adulthood,
partly due to the development of new therapies, but especially because of the tendency for earlier surgical
corrections. On the other hand, adult patients with non-corrected tetralogy of Fallot (TOF) are hard to find. The
objective of the present report was to describe a case of repair of TOF in an adult patient. Tetralogy of Fallot

occurs in approximately 400 per million live births

B.Acidosis metabolic

It is relatively common, particularly among acutely unwell/critical care patients. There are no
reliable figures for its overall incidence or prevalence in the population at large.

2.Prognosis
a. If it is not repaired, 70% of the children with this disorder die before the age of 10, but survival to adulthood
is possible 4, although after the age of 40, the rate of survival is about 3%.

Untreated, tetralogy of Fallot rapidly results in progressive right ventricular hypertrophy due to the
increased resistance on the right ventricle. This progresses to heart failure (dilated cardiomyopathy)
which begins in the right heart and often leads to left heart failure. Actuarial survival for untreated
tetralogy of Fallot is approximately 75% after the first year of life, 60% by four years, 30% by ten
years, and 5% by forty years.
B. This is largely dependent upon the underlying cause and severity of the illness in a given

patient. There is no doubt that metabolic acidosis can be life-threatening and carries
significant mortality and morbidity. Appropriate initial management and ongoing expert input
will improve the outlook for individual patients.
C

Causes of metabolic acidosis

There are many causes. They can be classified according to their pathophysiological origin,
as below. The table is not exhaustive but lists those that are most common or clinically
important to detect.
Increased acid load

Any cause of lactic acidosis e.g. heart failure,

drugs or toxins, inborn errors of metabolism.

Ketoacidosis due to diabetes, starvation or

alcohol excess.

Poisoning with substances that generate acid or

prevent its excretion, e.g.
o Methanol
o Salicylate
o Ethylene glycol

Excessive loss of gastrointestinal

bicarbonate

Diarrhoea

Fistulae of pancreas, biliary

tree or intestine

Urinary-gastrointestinal
diversion surgery

Cholestyramine

o Paraldehyde
o Iron
o Sulphur
o Toluene
o Biguanides
o Parenteral or hyperalimentary nutrition
o Isoniazid
o Ammonium chloride
o Cyclosporin
Impaired excretion of dietary acid load

Renal failure leading to impaired NH4+

production
Hypoaldosteronism in Type 4 Renal tubular

Excessive loss of renal bicarbonate

Type 2 (proximal) RTA

Acetazolamide
or
other
carbonic anhydrase inhibitors

acidosis (RTA)

Impaired excretion of H+ in Type 1 (distal)

RTA

Presentation

History
There are no specific symptoms of metabolic acidosis as such. Those that occur are due to the
effects of the metabolic derangement on the body, or may give clues to the underlying cause.
Patients may notice a subjective sensation of dyspnoea caused by stimulation of the
respiratory centre in an attempt to 'blow off' CO 2 and increase blood pH. Nausea, vomiting
and anorexia are frequently present, particularly in children. Metabolic acidosis occurring in
children is very rarely due to an inborn error of metabolism (conditions such as moderately
severe
gastroenteritis
being
more
common).
The following points should be covered in the history in an attempt to identify the underlying
cause:

Family history of any similar episodes of illness (may indicate inborn error of
metabolism).

Family or personal history of diabetes.

Symptoms of undiagnosed diabetes, e.g. polyuria, polydipsia or weight loss.

Symptoms of renal failure such as nocturia, polyuria, oliguria, pruritus and anorexia.

Recent history of urinary problems such as nephrolithiasis may indicate renal tubular
acidosis.

Recent history of severe or prolonged diarrhoea.

Recent nutritional status.

Alcohol intake.

Any history of deliberate or accidental ingestion of potentially toxic materials or

medicines.2

Occupational/DIY exposure to fumes or solvents.

Visual disturbances such as dimming, photophobia, scotomata or blindness may

indicate methanol poisoning. Blurred vision may occur with salicylate poisoning.

Tinnitus or vertigo may occur with salicylate poisoning.

Ask about any chest pain, palpitations, dyspnoea or oedema indicating possible
cardiovascular causes for lactic acidosis.

Recent history of confusion, headache or visual changes may indicate poisoning,

particularly with methanol or ethylene glycol.

Examination

Lethargy, stupor and progression to a state of coma may occur, particularly in cases of
poisoning. An intoxicated-appearing patient who has no smell of alcoholic drink on
their breath may have ingested ethylene glycol.

Check vital signs as hypotension may occur due to myocardial suppression in severe
acidaemia.

In undiagnosed renal failure there may be dryness of mouth, eyes and skin, scratch
marks on skin, pallor, drowsiness and fetor.

To detect diabetic ketoacidosis look for evidence of dehydration and smell the
patient's breath to detect the presence of ketones (which give off a musty/fruity odour
akin to pear-drops or nail-polish remover).

Look for signs of congestive cardiac failure that may be caused by the acidosis itself,
or suggest lactic acidosis as a cause, due to generalised hypoperfusion.

Listen for a pericardial rub which may indicate acute renal failure as the cause.

Tachypnoea is likely to be present.

The presence of tachypnoea without any history of pre-existing cardiorespiratory

disease to account for it should strongly suggest metabolic acidosis as the cause of the
illness.

Kussmaul's respiration may be noted where there is deep, slowly rhythmic breathing
that increases the minute tidal volume.

Children with chronic metabolic acidosis may suffer growth retardation and show
signs of rickets.

Neurological examination may reveal cranial nerve palsies in the case of ethylene
glycol poisoning.

Retinal oedema may be noted on fundoscopy in cases of methanol ingestion.

Differential diagnosis
The combination of clinical features of illness and arterial blood gas/plasma bicarbonate
results indicate the presence of a metabolic acidosis. Determination of its underlying cause,

as outlined in the investigation section below, is crucial to try and optimally treat and correct
the acidosis. As such there is no differential diagnosis, rather a list of possible underlying
causes to be refuted/confirmed as its cause. See the table above for a list of potential causes.
Investigations

The first indication of an acidotic problem may be the presence of a low serum
bicarbonate on routine U&E testing. This in itself is not enough to confirm an
acidosis. The presence of metabolic compensation of respiratory alkalosis, or a lab
error could account for a low plasma bicarbonate. Arterial blood gases must be
checked to determine the arterial blood pH and confirm the diminution of bicarbonate.
pH and pCO2 values must be interpreted carefully and a judgement made as to
whether this is a pure metabolic acidosis or a mixed acid-base disorder.3 The base
excess or base deficit is usually given as a part of the ABG result and allows a
determination of the overall severity of the acidosis, particularly where respiratory
compensation complicates the picture.

U&Es help to determine the cause of the acidosis by allowing the calculation of the
anion gap as below:

Anion Gap=([Na mmol/L] + [K mmol/L]) ([HCO3 mmol/L] + [Cl mmol/L])

The normal anion gap varies between labs but is usually 1012 mmol/L; it can be as
high as 20 mmol/L if potassium is included in the equation, in some labs. It is a
measure of the organic anions not routinely detected by analyser machines. Where the
anion gap is elevated it indicates the presence of an organic acid causing the acidosis,
for example acetoacetic acid and beta-hydroxybutyric acid in diabetic ketoacidosis.
The anion gap helps to determine the likely cause of the acidosis as outlined below.

Correlation between anion gap and causes of metabolic acidosis

Elevated anion-gap metabolic acidoses
Normal anion-gap metabolic acidoses
(hyperchloraemic acidosis)
Lactic acidosis caused by L-lactate, due to
Gastrointestinal bicarbonate loss,
state
of
hypoperfusion,
carbon
monoxide/cyanide poisoning, biguanide
e.g. diarrhoea, pancreatic or
toxicity. D-Lactataemia in short bowel
intestinal fistulae
syndrome.
Renal bicarbonate loss, e.g. Type
Diabetic or alcoholic ketoacidosis caused by
2 RTA
acetoacetate/beta-hydroxybutyrate.
Some
causes
of
renal
Renal failure caused by urate, hippurate,
failure/impairment
sulphate and phosphate.
Hypoaldosteronism in Type 4
Salicylate poisoning.
RTA

Methanol or formaldehyde poisoning caused

by formate.

Hyperventilation

Ethylene glycol poisoning

glycolate and oxalate.

caused

by

Paraldehyde poisoning caused by a variety

of organic acids.

Sulphur poisoning caused by sulphate.

Pyroglutamic
oxoprolate.

acidaemia

caused

Ingestion of ammonium chloride

Use of carbonic
inhibitors

Parenteral or hyperalimentation
feeding

Partially-treated
ketoacidosis

by 5-

Rhabdomyolysis caused by direct proton

release from lysed muscle cells.

anhydrase

diabetic

Raised AG can be due to low levels of unmeasured cations, e.g. hypomagnesaemia,

hypocalcaemia. AG correlates closely with albumin levels and must be adjusted in
hypoalbuminaemia. AG can be decreased by raised unmeasured cations such as in
hypercalcaemia, hypermagnesaemia, lithium toxicity. Hyperproteinaemia, hyperlipidemia and
hyperglycaemia can affect AG by falsely depressing measured sodium level. Bromide
poisoning may cause Br ions to be mistaken for Cl ions by the autoanalyser causing
inappropriate depression of the AG.

Further general investigations

Elevation of urea and creatinine will indicate that renal failure is the likely cause.

Hyperkalaemia often accompanies a normal-anion-gap acidosis, and some cases of

acute
renal
failure.
Potassium may also be elevated in diabetic ketoacidosis but usually minimally so for
the degree of acidosis.

Plasma and urinary glucose and ketones need to be checked to look for evidence of
diabetic ketoacidosis.

Alcoholic ketoacidosis will show evidence of ketone formation without grossly

elevating plasma glucose in most cases. A random alcohol level or expired breath
alcometer reading may help to make the diagnosis, along with details from the history.

FBC should be checked but is usually non-specific and unhelpful, except in the case
of severe anaemia, where it is likely that lactic acidosis is the cause, or grossly
elevated WCC indicating sepsis or haematological neoplasm.

LFTs should be checked to look for evidence of chronic liver disease, indicating
alcohol abuse as a likely aetiology.

Send any appropriate samples for culture, particularly blood and urine if sepsis
leading to lactic acidosis is possible.

ECG helps to detect arrythmias.

Consider CXR to look for evidence of infection/cardiac failure or ingested iron/other

radio-opaque toxins.

Investigations for specific causes

Osmotic gap. This is the difference between the measured osmolality and the
calculated osmolality. Calculated osmolality is worked out using the formula below:
Calculated osmolality = 2[Na mmol/L] + [Urea mmol/L] + [glucose mmol/L]

Where the osmotic gap is elevated, it indicates a disparity between the actual
osmolality (measured by the laboratory) and the calculated osmolality. This indicates
the presence of other osmotically active solutes which are not taken into account in
the calculated osmolality. It is normally less than 10-15mOsmol/Kg (see local lab for
range). It is elevated by methanol or ethylene glycol ingestion.

Plasma lactate, where lactic acidosis is a potential cause. Values >2 mmol/L indicate
hyperlactataemia and >5 mmol/L indicate definite lactic acidosis.

Plasma salicylate levels. Levels >4050 mg/dl indicate salicylate toxicity. >100 mg/dl
indicates severe toxicity.

Iron levels if there is a suspicion of deliberate or accidental overdose. Levels >300

mg/dl are considered toxic. An abdominal x-ray may be of use if there is suspected
ingestion of iron tablets.

Urine microscopy may show the presence of needle-shaped oxalate crystals which is
indicative of ethylene glycol toxicity. Usually urine pH will be <5.05.5. Alkaline
urine in the face of acidosis is usually caused by Type 1 renal tubular acidosis or
salicylate poisoning.

Management
Patients with metabolic acidosis are often very ill and prone to rapid deterioration. Make
your assessment of their condition and its likely cause as quickly and calmly as possible.
Arrange any investigations, as above, that will help to reveal its aetiology. Ask for advice
from senior colleagues/other appropriate specialties early in the course of the presentation.

General measures

Put patient in resuscitation area, or transfer to high dependency area as soon as

feasible.

Put patient on ECG monitor, SaO2 monitor and BP/HR monitor.

In patients who are clinically unwell and have deteriorating SaO 2 levels or conscious
levels, consider intubation and assisted ventilation, after taking senior
A&E/medical/anaesthetic advice.

Get large-bore IV access (central venous line may be needed) and rehydrate
aggressively. Use colloids if necessary.

Consider catheterisation to monitor urine output and obtain urine for analysis.

If there is any possibility of drug or toxin ingestion give initial therapies such as
activated charcoal/chelating agents/emetics, dependent on the specific compound
ingested and latest local guidelines for poisoning.

Liaise with local or national toxicology/poisoning services if there has been ingestion
of a potentially dangerous substance.

Obtain specialist input (usually on-call general-medical team initially) as soon as

possible.

Correction of acidosis
Treatment of the underlying cause is the aim. Use of bicarbonate infusions is not
recommended as it can lead to a fatal outcome. It should be used only where advised in cases
of poisoning.

Specific therapy for the underlying cause

This is the most important and efficient way to correct the acidosis and improve the patient's
outlook. Toxicological/general medicine/renal medicine expertise should be engaged to offer
specific therapy for the identified underlying problem.
Complications
The major problem is suppression of myocardial contractility and unresponsiveness to
catecholamines caused by the acidaemic state. This may lead to a vicious cycle of
hypoperfusion, worsening lactic acidosis and further cardiac suppression, causing multiorgan failure. If pH is <7.17.2 then cardiac arrythmias are likely.
Prognosis
This is largely dependent upon the underlying cause and severity of the illness in a given
patient. There is no doubt that metabolic acidosis can be life-threatening and carries
significant mortality and morbidity. Appropriate initial management and ongoing expert input
will improve the outlook for individual patients.
Asidosis respirasi
Respiratory acidosis is a clinical disturbance due to alveolar hypoventilation. Production of
carbon dioxide occurs rapidly, and failure of ventilation promptly increases the partial arterial

pressure of carbon dioxide (PaCO2). The normal reference range for PaCO2 is 36-44 mm Hg.
Alveolar hypoventilation leads to an increased PaCO 2 (ie, hypercapnia). The increase in
PaCO2, in turn, decreases the bicarbonate (HCO3 -)/PaCO2, decreasing the pH. Hypercapnia
and respiratory acidosis ensue when impairment in ventilation occurs and the removal of
carbon dioxide by the lungs is less than the production of carbon dioxide in the tissues.
Respiratory acidosis can be acute or chronic. In acute respiratory acidosis, the PaCO 2 is
elevated above the upper limit of the reference range (ie, >45 mm Hg) with an accompanying
acidemia (ie, pH <7.35). In chronic respiratory acidosis, the PaCO 2 is elevated above the
upper limit of the reference range, with a normal or near-normal pH secondary to renal
compensation and an elevated serum bicarbonate value (ie, >30 mm Hg).
Acute respiratory acidosis is present when an abrupt failure of ventilation occurs. This failure
in ventilation may be caused by depression of the central respiratory center by cerebral
disease or drugs, an inability to ventilate adequately owing to a neuromuscular disease
(eg, myasthenia gravis, amyotrophic lateral sclerosis, Guillain-Barr syndrome, muscular
dystrophy), or airway obstruction related to asthma or chronic obstructive pulmonary
disease (COPD).
Chronic respiratory acidosis may be secondary to many disorders, including COPD.
Hypoventilation in COPD involves multiple mechanisms, including decreased responsiveness
to hypoxia and hypercapnia, increased ventilation-perfusion mismatch leading to increased
dead space ventilation, and decreased diaphragmatic function due to fatigue and
hyperinflation.
Chronic respiratory acidosis also may be secondary to obesity-hypoventilation syndrome (ie,
pickwickian syndrome), neuromuscular disorders such as amyotrophic lateral sclerosis, and
severe restrictive ventilatory defects as observed in interstitial fibrosis and thoracic
deformities.
Lung diseases that primarily cause abnormalities in alveolar gas exchange usually do not
cause hypoventilation; however, they tend to cause stimulation of ventilation and hypocapnia
secondary to hypoxia. Hypercapnia only occurs if severe disease or respiratory muscle
fatigue is present.
Pathophysiology

Metabolism rapidly generates a large quantity of volatile acid (carbon dioxide) and
nonvolatile acid. The metabolism of fats and carbohydrates leads to the formation of a large
amount of carbon dioxide. The carbon dioxide combines with water to form carbonic acid (H 2
CO3). The lungs excrete the volatile fraction through ventilation, and acid accumulation does
not occur. A significant alteration in ventilation that affects elimination of carbon dioxide can
cause a respiratory acid-base disorder. The PaCO 2 is normally maintained within the range of
35-45 mm Hg.1,2

Alveolar ventilation is under the control of the central respiratory centers, which are located
in the pons and the medulla. Ventilation is influenced and regulated by chemoreceptors for
PaCO2, PaO2, and pH located in the brainstem, as well as by neural impulses from lungstretch receptors and impulses from the cerebral cortex. Failure of ventilation quickly
increases the PaCO2.
In acute respiratory acidosis, the body's compensation occurs in 2 steps. The initial response
is cellular buffering that occurs over minutes to hours. Cellular buffering elevates plasma
bicarbonate values, but only slightly, approximately 1 mEq/L for each 10-mm Hg increase in
PaCO2. The second step is renal compensation that occurs over 3-5 days. With renal
compensation, renal excretion of carbonic acid is increased and bicarbonate reabsorption is
increased.
The expected change in serum bicarbonate concentration in respiratory acidosis can be
estimated as follows:

Acute respiratory acidosis: Bicarbonate increases 1 mEq/L for each 10-mm Hg rise in PaCO 2.

Chronic respiratory acidosis: Bicarbonate increases 3.5 mEq/L for each 10-mm Hg rise in
PaCO2.

The expected change in pH with respiratory acidosis can be estimated with the following
equations:

Acute respiratory acidosis: Change in pH = 0.008 X (40 - PaCO2)

Chronic respiratory acidosis: Change in pH = 0.003 X (40 - PaCO2)

Respiratory acidosis does not have a great effect on electrolyte levels. Some small effects
occur in calcium and potassium levels. Acidosis decreases binding of calcium to albumin and
tends to increase serum ionized calcium levels. In addition, acidemia causes an extracellular
shift of potassium, but respiratory acidosis rarely causes clinically significant hyperkalemia.
Mortality/Morbidity

The morbidity and mortality of respiratory acidosis depends on the underlying cause of the
respiratory acidosis, associated conditions, the patient's compensatory mechanisms,
and effectiveness of medical care.

Clinical
History

The clinical manifestations of respiratory acidosis often are those of the underlying disorder.
Manifestations vary depending on the severity of the disorder and on the rate of development
of hypercapnia. Mild-to-moderate hypercapnia that develops slowly usually has minimal
symptoms.

Patients may be anxious and may complain of dyspnea. Some patients may have disturbed
sleep and daytime hypersomnolence. As the PaCO2 increases, the anxiety may progress to
delirium, and patients become progressively more confused, somnolent, and obtunded. This
condition is sometimes referred to as carbon dioxide narcosis.
Physical

The findings upon physical examination in patients with respiratory acidosis usually are
nonspecific and are related to the underlying illness or the cause of the respiratory acidosis.
Thoracic examination of patients with obstructive lung disease may demonstrate diffuse
wheezing, hyperinflation (ie, barrel chest), decreased breath sounds, hyperresonance on
percussion, and prolonged expiration. Rhonchi also may be heard.
Cyanosis may be noted if accompanying hypoxemia is present. Digital clubbing may indicate
the presence of a chronic respiratory tract disease or other organ system disorders.
The patient's mental status may be depressed if he or she has severe elevations of PaCO 2.
Patients may have asterixis, myoclonus, and seizures.
Papilledema may be found during the retina examination. Conjunctival and superficial facial
blood vessels also may be dilated.
Causes

Respiratory acidosis may occur due to a variety of etiologies, including the following:

Chronic obstructive pulmonary disease - Emphysema, severe asthma 3,4 , chronic bronchitis

Neuromuscular diseases - Amyotrophic lateral sclerosis, diaphragm dysfunction and paralysis,

Guillain-Barr syndrome, myasthenia gravis, muscular dystrophy

Chest wall disorders - Severe kyphoscoliosis; status post thoracoplasty; flail chest; less
commonly, ankylosing spondylitis, pectus excavatum, 5 or pectus carinatum

Obesity-hypoventilation syndrome

Obstructive sleep apnea

CNS depression - Drugs (eg, narcotics, barbiturates, benzodiazepines, other CNS

depressants), neurologic disorders (eg, encephalitis, brainstem disease, trauma), primary
alveolar hypoventilation

Other lung and airway diseases - Laryngeal and tracheal stenosis

Lung-protective ventilation in ARDS

ASIDOSIS
PENDAHULUAN
Asidosis adalah suatu keadaan dimana adanya peningkatan asam didalam darah yang
disebabkan oleh berbagai keadaan dan penyakit tertentu yang mana tubuh tidak bisa
mengeluarkan asam dalam mengatur keseimbangan asam basa. Hal ini penting untuk
menjaga keseimbangan fungsi sistem organ tubuh manusia. Gangguan keseimbangan ini
dapat dikelompokkan dalam dua kelompok besar yaitu metabolik dan respiratorik. Ginjal dan
paru merupakan dua organ yang berperan penting dalam pengaturan keseimbangan ini.
( Siregar P et. al, 2001 )
PATOGENESIS
Pada keadaan Asidosis yang berperan adalah sistem buffer (penyangga) pada referensi ini
akan dibahas tentang sistem buffer bikarbonat. Sistem penyangga bikarbonat terdiri dari
larutan air yang mengandung bikarbonat yang terdiri dari larutan air yang mengandung dua
zat yaitu asam lemak (H2CO3) dan garam bikarbonat seperti NaHCO3.
H2CO3 dibentuk dalam tubuh oleh reaksi CO2 dengan H2O.
CO2 + H2O <-> H2CO3
Reaksi ini lambat dan sangat sedikit jumlah H2CO3 yang dibentuk kecuali bila ada enzim
karbonik anhidrase. Enzim ini terutama banyak sekali di dinding alveol
paru dimana CO2 dilepaskan, karbonik anhidrase juga ditemukan di sel-sel epitel tubulus
ginjal dimana CO2 bereaksi dengan H2O untuk membentuk H2CO3
H2CO3 berionisasi secara lemah untuk membentuk sejumlah kecil H+ dan HCO3H2CO3 <-> H+ + HCO3Komponen kedua dari sistem yaitu garam bikarbonat terbentuk secara dominan sebagai
Natrium Bicarbonat (NaHO3) dalam cairan ekstraseluler. NaHCO3 berionisasi hampir secara
lengkap untuk membentuk ion-ion bicarbonat (HCO3-) dan ion-ion natrium (Na+) sebagai
berikut :
NaHCO3 <-> Na+ + HCO3Sekarang dengan semua sistem bersama-sama, kita akan mendapatkan sebagai berikut :
CO2 + H2O <-> H2CO3 <-> H+ + HCO3- + Na+

Akibat disosiasi H2CO3 yang lemah, konsentrasi H+ menjadi sangat kuat bila asam kuat
seperti HCl ditambahkan ke dalam larutan penyangga bicarbonat, peningkatan ion hidrogen
yang dilepaskan oleh asam disangga oleh HCO3 :
H + + HCO3- H2CO3 CO2 + H2O
Sebagai hasilnya, lebih banyak H2CO3 yang dibentuk. Meningkatkan produksi CO2 dan H2O.
Dari reaksi ini kita dapat melihat bahwa ion hidrogen dari asam kuat HCl, bereaksi dengan
HCO3- untuk membentuk asam yang sangat lemah yaitu H2CO3 yang kemudian membentuk
CO2 dan H2O. CO2 yang berlebihan sangat merangsang pernapasan yang mengeluarkan CO2
dari cairan ekstraseluler. Ini berpengaruh terjadinya asidosis pada tubuh.
ETIOLOGI
Asidosis Metabolik
Asidosis metabolik dapat disebabkan oleh beberapa penyebab umum seperti :
1. Kegagalan ginjal untuk mengekresikan asam metabolik yang normalnya dibentuk di tubuh.
2. Pembentukan asam metabolik yang berlebihan dalam tubuh.
3. Penambahan asam metabolik kedalam tubuh melalui makanan
4. Kehilangan basa dari cairan tubuh (faal)

Disini penulis akan sedikit membahas beberapa penyebab yang sering terjadi pada keadaan
asidosis metabolik :
- Asidosis di Tubulus Ginjal
Akibat dari gangguan ekresi ion Hidrogen atau reabsorbsi bikarbonat oleh ginjal atau keduaduanya. Gangguan reabsorbsi bikarbonat tubulus ginjal menyebabkan hilangnya bicarbonat
dalam urine atau ketidakmampuan mekanisme sekresi Hidrogen di tubulus ginjal untuk
mencapai keasaman urin yang normal menyebabkan ekresi urin yang alkalis.
-

Diare

Diare berat mungkin merupakan penyebab asidosis yang paling sering. Penyebabnya adalah
hilangnya sejumlah besar natrium bicarbonat ke dalam feses, sekresi gastrointestinal secara
normal mengandung sejumlah besar bicarbonat dan diare ini menyebabkan hilangnya ion
bicarbonat dari tubuh. Bentuk asidosis metabolik ini berlangsung berat dan dapat
menyebabkan kematian terutama pada anak-anak.
-

Diabetes Melitus

Diabetes melitus disebabkan oleh tidak adanya sekresi insulin oleh pankreas yang
menghambat penggunaan glukosa dalam metabolisme.Ini terjadi karena adanya pemecahan
lemak menjadi asam asetoasetat dan asam ini di metabolisme oleh jaringan untuk
menghasilkan energi, menggantikan glukosa. Pada DM yang berat kadar Asetoasetat dalam
darah meningkat sangat tinggi sehingga menyebabkan asidosis metabolik yang berat.
-

Penyerapan Asam

Jarang sekali sejumlah besar asam diserap dari makanan normal akan tetapi asidosis
metabolik yang berat kadang-kadang dapat disebabkan oleh keracuan asam tertentu antara
lain aspirin dan metil alkohol.
-

Gagal Ginjal Kronis

Saat fungsi ginjal sangat menurun terdapat pembentukan anion dari asam lemak dalam cairan
tubuh yang tidak eksresikan oleh ginjal. Selain itu penurunan laju filtrasi glomerulus
mengurangi eksresi fosfat dan NH4+ yang mengurangi jumlah bikarbonat.
( Guyton & Hall, 1997 )
Faktor Resiko Asidosis Metabolik ( Defisit HCO3- )
1. Kondisi dimana banyak plasma dengan asam metabolik (Gangguan ginjal, DM)
2. Kondisi tejadi penurunan bikarbonat (diare)
3. Cairan infus yang berlebihan. (NaCl)
4. Napas berbau
5. Napas Kussmaul (dalam dan cepat)
6. Letargi
7. Sakit kepala
8. Kelemahan
9. Disorientasi

Gejala Klinik
Asidosis Respiratorik
Keadaan ini timbul akibat ketidakmampuan paru untuk mengeluarkan CO2 hasil metabolisme
(keadaan hipoventilasi). Hal ini menyebabkan peningkatan H2CO3 dan konsentrasi ion
hidrogen sehingga menghasilkan asidosis.

Beberapa masalah respiratorik dibagi berdasarkan sebabnya :

1. Penurunan pernapasan
Penurunan pernapasan melibatkan perubahan fungsi neuron dalam menstimulus inhalasi dan
ekhalasi. Neuron mengurangi pada tingkat sel tubuh melalui zat/agen kimia dan kerusakan
fisik. Penurunan kimia pada neuron dapat terjadi sebagai hasil agen anastesi, obat-obatan
(narkotik) dan racun dimana merintangi darah menuju ke otak dan langsung menghalangi
depolarisasi. Disamping itu ketidakseimbangan elektrolit (hiponatrium, hiperkalsemia dan
hiperkalami) juga secara lambat menghalangi depolarisasi neural. Akibat neuron
respiratorik juga akan mengurangi keadaan fisik. Trauma sebagai hasil langsung
kerusakan fisik untuk neuron respirasi atau menimbulkan hypoksia sampai iskemik yang
dapat mengganggu atau menghancurkan kemampuan neuron untuk membangkitkan dan
mengirimkan impuls ke otot skeletal yang membantu dalam respirasi. Neuron respirasi dapat
rusak atau hancur secara tidak langsung apabila terdapat masalah di area otak karena
meningkatnya tekanan intrakranial. Meningkatnya tekanan intrakranial ini karena adanya
edema jaringan, dimana menekan pusat pernapasan (batang otak).
Trauma spinal cord, penyakit tertentu seperti polio adalah sebab yang aktual bagi kerusakan
diaxon dan penyakit lain seperti mistenia gravis, dan syndrom Guillain-Barre yang
mengganggu tranmisi impuls nervous ke otot skeletal)
2. Inadequatnya ekspansi dada
Karena ekspansi ini penting untuk mengurangi tekanan di dalam rongga dada sehingga terjadi
pernapasan. Beberapa kondisi membatasi ekspansi dada sehingga menghasilkan inadequatnya
pertukaran gas walaupun jaringan paru sehat dan pusat pesan sudah dimulai dan transmisi
yang tepat. Beberapa orang mengalami masalah dalam ekspansi dada dapat mencukupi
pertukaran gas selama periode istirahat sehingga retensi CO2 tidak terjadi pada waktu itu.
Bagaimanapun meningkatnya aktivitas atau kerusakan pada jaringan paru menghasilkan
permintaan untuk pertukaran gas dimana seseorang tidak dapat memenuhinya, hasilnya
acidemia. Tidak adekuatnya ekspansi dada dapat dihasilkan dari trauma skeletal atau
deformitas, kelemahan otot respirasi. Masalah skeletal yang membatasi perpindahan
pernapasan dalam dinding dada jika terdapat kerusakan tulang atau malformasi tulang yang
menyebabkan distorsi dalam fungsi dada. Struktur tulang dada yang tidak berbentuk serasi
dapat membentuk deformasi pada rongga dada dan mencegah penuhnya ekspansi pada satu
atau kedua paru. Deformitas skeletal mungkin congenital: hasil dari kesalahan pertumbuhan
tulang ( seperti skoliosis, osteodistropii renal, osteogenesis imperfecta dan syndrom Hurlers)
atau hasil yang tidak seimbang dari degenerasi jaringan tulang (osteoporosis, metastase sel
kanker).
Kondisi kelemahan otot respirasi berhubungan dengan ketidakseimbangan elektrolit dan
kelelahan.

3. Obstruksi jalan napas

Pencegahan perpindahan masuk dan keluarnya udara pada paru melalui bagian atas dan
bawah pada obstruksi jalan napas dapat menimbulkan pertukaran gas yang tidak efektif,
retensi CO2 dan acidemia. Jalan napas bagian atas dan bawah dapat terobstruksi secara
internal dan eksternal. Kondisi eksterna yang menyebabkan obstruksi jalan napas atas
termasuk tekanan yang kuat pada daerah leher, pembesaran nodus lympa regional. Sedangkan
kondisi internal yang menyebabkan obstruksi jalan napas atas termasuk masuknya benda
asing pada saat bernapas, konstriksi otot halus bronkial dan pembentukan edema pada
jaringan luminal.
Obstruksi jalan napas bagian bawah terjadi melalui kontriksi otot halus, pembentukan
jaringan luminal, pembentukan lendir yang berlebihan. Kondisi umum yang berhubungan
dengan obstruksi jalan napas bagian bawah yaitu karena terlalu lama menderita penyakit
inflamasi (bronchitis, emphysema dan asma) dan dan masuknya bahan-bahan iritan seperti
asap rokok, debu batu bara, serat asbes, serat kapas, debu silikon dan beberapa partikel yang
mencapai jalan napas bagian bawah.
4. Gangguan difusi alveolar-kapiler
Pertukaran gas pulmonal terjadi oleh difusi di persimpangan alveolar dan membran kapiler.
Beberapa kondisi dimana mencegah atau mengurangi proses difusi karena dapat meretensi
CO2 dan terjadi asidemia. Masalah difusi dapat terjadi pada membran alveolar, membran
kapiler atau area diantara keduanya.
Asidosis respiratorik sering terjadi akibat kondisi patologis yang merusak pusat pernapasan
atau yang menurunkan kemampuan paru untuk mengeliminasikan CO 2. Ada beberapa hal
yang menyebabkan keadaan asidosis respiratorik yaitu :
- gangguan sentral pada pusat pernapasan.
- penyakit otot-otot bantu pernapasan

misal mistenia gravis, sindrom

Guillain- Barre dan akibat obat yang merelaksasi otot.

- gangguan eksfisitas saluran napas seperti fibrosis pulmonal, penyakit
intestinal

paru.

- obstruksi (empisema, asma, bronkitis, bronkhiolitis).

Faktor Resiko Asdidosis Respiratorik yang lain :
1. Kondisi paru yang akut dimana merubah O 2 atau CO2 pada saat terjadi pertukaran gas di
alveolar (seperti pnemonia, edema pulmonar akut, aspirasi pada tubuh luar, tenggelam)

2. Penyakit paru kronik (asma, kista fibrosis atau empisema)

3. Overdosis pada narkotik atau sedatif sehingga menekan tingkat dan kedalaman pernapasan
4. Cidera kepala sehingga mempengaruhi pusat pernapasan.

Tanda Klinik ( Akut )

1. Meningkatnya nadi dan tingkat pernapasan
2. Pernapasan dangkal.
3. Dyspnea
4. Pusing
5. Convulsi
6. Letargi

Tanda Klinik ( Kronik )

1. Kelemahan
2. Sakit kepala

PENATALAKSANAAN ASIDOSIS
Pengobatan yang paling baik untuk asidosis adalah mengoreksi keadaan yang telah
menyebabkan kelainan, seringkali pengobatan ini menjadi sulit terutama pada penyakit kronis
yang menyebabkan gangguan fungsi paru atau gagal ginjal.
Untuk menetralkan kelebihan asam sejumlah besar natrium bicarbonat dapat diserap melalui
mulut. Natrium bicarbonat diabsorbsi dari traktus gastroinstestinal ke dalam darah dan
meningkatkan bagian bicarbonat pada sistem penyangga bicarbonat sehingga meningkatkan
pH menuju normal. Natrium bicarbonat dapat juga diberikan secara intravena. Untuk
pengobatan asidosis respiratorik dapat diberikan O 2 dan juga obat-obatan yang bersifat
broncodilator.
Intervensi keperawatan yang bisa dilakukan pada Asidosis Metabolik :
1. Monitor nilai Arterial Gas Darah
2. Jika diperintah berikan IV sodium bicarbonat
3. Koreksi masalah pokok yang terjadi.

Intervensi keperawatan yang bisa dilakukan pada Asidosis Respiratorik :

1. Perbaiki ventilasi pernapasan ( melakukan dilator bronkial, antibiotik, O 2 sesuai perintah.

2. Jaga keadequatan hidrasi (2 3 L cairan perhari)
3. hati-hati dalam mengatur ventilator mekanik jika digunakan.
4. Monitor intake dan output cairan, TTV, arteri gas darah dan pH.

PENGUKURAN KLINIS DAN ANALISIS ASIDOSIS

Seseorang dapat membuat diagnosa dari analisis terhadap tiga pengukuran dari suatu contoh
darah arterial : pH, konsentrasi bikarbonat plasma dan PCO2.
- Dengan memeriksa pH seseorang dapat menentukan apakah ini bersifat
asidosis jika nilai pH kurang dari 7,4.
- Langkah kedua adalah memeriksa PCO2 plasma dan konsentrasi bicarbonat. Nilai normal
untuk PCO2 adalah 40 mmHg dan untuk bicarbonat 24 mEq/L Bila gangguan sudah ditandai
sebagai asidisis dan PCO2 plasma meningkat. Oleh karena itu nilai yang diharapkan untuk
asidosis respiratorik sederhana adalah penurunan pH plasma, peningkatan PCO 2 dan
peningkatan konsentrasi bicarbonat plasma setelah kompensasi ginjal sebagian.
Untuk asidosis metabolik akan terdapat juga penurunan pH plasma. Gangguan utama adalah
penurunan konsentrasi bicarbonat plasma. Oleh karena itu pada asidosis metabolik, seseorang
dapat mengharapkan nilai pH yang rendah. Konsentrasi bicarbonat plasma rendah dan
penurunan PCO2 setelah kompensasi respiratorik sebagian.

Transposition of the Great Arteries

Synonyms: TGA, complete transposition of the great arteries, d-TGA, simple transposition,
ventriculoarterial
discordance
The aorta and pulmonary artery are transposed so that the aorta arises from the right ventricle
and the pulmonary artery arises from the left ventricle. The aetiology is unknown and
inheritance is presumed to be multifactorial. Transposition is often associated with other heart
defects, e.g. ventricular septal defect, left ventricular outflow obstruction, atrial septal defect,
patent ductus arteriosus. The presence or absence of associated cardiac anomalies determines
the presentation and management.
Epidemiology

Transposition is the most common cyanotic congenital heart lesion presenting in the neonate.

The overall annual incidence is 20-30 per 100,000 live births. 1

It is more common in males than females, with a ratio of about 3:1.

Maternal factors associated with an increased risk include rubella or other viral illness during
pregnancy, alcoholism, maternal age over 40 and diabetes.

Transposition is rarely associated with syndromes or extracardiac malformations.

Presentation

Most affected infants present with cyanosis, most often within the first 2 or 3 days, and many
require resuscitation.2Some cases are more mild in presentation and not diagnosed until the
end of the first week of life or beyond.

Those with a large ventricular septal defect or patent ductus arteriosus may not be diagnosed
until several weeks of age.

Infants may develop breathlessness and heart failure over the first 3-6 weeks as pulmonary
blood flow increases.

If there is a ventricular septal defect and left ventricular outflow tract obstruction, the
presentation may be similar to that of an infant with Fallot's tetralogy.

Signs

Cyanosis and may also be breathless with tachycardia and tachypnoea.

Heart sounds are often normal with no audible murmurs.

In patients with a ventricular septal defect there may be a systolic murmur, which increases in
intensity as the pulmonary vascular resistance falls.

An ejection systolic murmur is usually present in those with left ventricular outflow tract
obstruction.

Differential diagnosis

Non-cardiac causes of a severely ill neonate, e.g. infection, respiratory problems (such as
respiratory distress syndrome, meconium aspiration, pneumothorax, pneumonia, congenital
diaphragmatic hernia)

Other causes of congenital heart disease, especially:

o

Pulmonary atresia

Fallot's tetralogy

Total anomalous pulmonary venous connection

Tricuspid atresia

Truncus arteriosus

Investigations

Antenatal diagnosis by ultrasound scanning results in better clinical status before surgery and
improved postoperative outcome.2

Chest X-ray may appear normal in newborns with transposition of the great arteries (TGA)
and intact ventricular septum but may demonstrate the classic 'egg on a string' appearance
(heart is slightly enlarged and appears like an egg lying on its side, narrow vascular pedicle
because aorta and pulmonary artery lie one in front of the other, and increased vascular lung
markings).

With an associated ventricular septal defect, the chest X-ray usually shows cardiomegaly with
increased pulmonary arterial vascular markings.

Initial investigations also include pulse oximetry, ECG and assessment of an unwell baby, e.g.
infection screen, full blood count, renal function, electrolytes.

Echocardiogram images are diagnostic of transposition and associated anatomic lesions.

Cardiac catheterisation may be needed if echocardiogram does not adequately demonstrate

the anatomical abnormality.

Management

Palliative treatment with prostaglandin E1 infusion to maintain ductal patency and balloon
atrial septostomy are usually required soon after birth. 3

All patients require antibiotic prophylaxis prior to dental and indicated surgical procedures in
order to reduce the risk of subacute bacterial endocarditis.

Surgical

Cardiac catheterisation and balloon atrial septostomy are used to increase the atrial level shunt
and to improve mixing.

The definitive corrective procedure is the arterial switch operation, which has been found to
have similar mortality and less morbidity than atrial (Mustard or Senning) repairs. 4

Most fullterm neonates with uncomplicated transposition of the great arteries (TGA) can
undergo an arterial switch procedure in one operation, with minimal mortality.

Complications

Congestive heart failure

Arrhythmias

Right ventricular dysfunction in long-term survivors

Eisenmenger's syndrome

Polycythaemia and hyperviscosity syndrome

Seizures may occur in about 5% of patients before surgery2

Thrombocytopenia

Prognosis

The mortality rate in untreated patients is approximately 30% in the first week, 50% in the
first month, and 90% by the end of the first year.

Death is usually due to anoxia, acidosis, heart failure and complications associated with
polycythaemia, including thromboembolic events.

The overall survival rate following arterial switch operation is approximately 90% at 15 years
of age. However, exercise performance, cognitive function and quality of life may be
impaired.3

Low gestational age and a high preoperative lactate are the most important predictors of poor
developmental outcome.2