Tricuspid atresia

Dr. Rajkumar Ghumare Moderator : Dr. Savitri Srivastava

Introduction

An uncommon congenital cardiac malformation. It was first described by Kreysig in 1817 . Approximate incidence 5 per 1 lakh live births. Although tricuspid atresia usually occurs as an isolated defect, multiple cardiac anomalies have been reported in >20% of patients. The cause of this defect is unknown.

Definition

Tricuspid atresia is defined as a complete absence of the tricuspid valve with no direct communication between the right atrium and right ventricle.
This defect invariably leads to some degree of hypoplasia of the right ventricle. There is an obligatory right-to-left shunt at the atrial level, either through a widely patent foramen ovale or an ASD. There also must be a communication between the systemic and the pulmonary circulation, usually in the form of a VSD. Occasionally, however, there is associated pulmonary atresia, and pulmonary blood flow is supplied by a patent ductus arteriosus and / or aortopulmonary collaterals.

Tricuspid atresia

Classification
TYPE I : Normally related great arteries (70 80 %) Ia : Intact Vent Septum with Pulm Atresia PBF Ib : Small VSD with PS Ic : Large VSD without PS PBF

TYPE II : Transposition of great arteries (12 25 %) IIa : VSD with Pulmonary Atresia PBF IIb : VSD with PS IIc : VSD without PS PBF
TYPE III : Transposition or malposition of great arteries , associated complex lesions ( 3 6 %)

Classification
Ia Ib Ic

IIa

IIb

IIc

Morphology

The atretic tricuspid valve is represented by a dimple in the floor of the right atrium. The resulting membrane is usually muscular but may be fibrous.
The interatrial communication is usually a widely patent foramen ovale but may be a secundum ASD or, rarely, an ostium primum defect . When a VSD is present, it is usually perimembranous but may be muscular septum or rarely as a component of an AVSD. In most patients with tricuspid atresia, the inlet portion of the RV is absent and RV is composed largely of the infundibular portion and an incompletely formed trabecular portion

Morphology

Morphology

In patients with associated transposition, VSD is almost always present and is usually large. Aorta is anterior arising from RV and ascending aorta is smaller than normal. Associated coarctation may be seen. Pulmonary artery arise from LV , is placed posterior and its main branches are large. LA and LV show considerable enlargement and hypertrophy. 10-15 % have associated persistent left superior vena cava.

Physiology

Systemic venous return mixes completely with pulmonary venous return in the left atrium and subsequently flows across the mitral valve into the left ventricle. In patients with a VSD and a patent right ventricular outflow tract, blood is ejected directly into the normally arising aorta as well as through the VSD and the hypoplastic right ventricle into the pulmonary artery. The amount of pulmonary blood flow depends on the size of the VSD and the presence or absence of obstruction in RVOT. As the pulmonary vascular resistance decreases in the newborn infant, pulmonary overcirculation may develop.

Physiology

Cyanosis is invariably present to some extent because of complete mixing of the pulmonary and systemic venous returns. The degree of cyanosis depends on the magnitude of pulmonary blood flow. Pulmonary overcirculation will not occur when the pulmonary vascular resistance decreases in patients with a restrictive VSD or some form of RVOT obstruction, and the degree of cyanosis is likely to be greater. In patients with an intact ventricular septum or pulmonary valve atresia, pulmonary blood flow is supplied through a patent ductus arteriosus and / or arterial collaterals. The size of the ductus arteriosus and/or collaterals will determine the degree of pulmonary blood flow and consequently the degree of cyanosis.

Physiology
PBF

PBF

Tricuspid atresia with TGA Physiology

VSD is usually large and non-obstructive. Pulmonary overcirculaton occurs within few weeks as obstruction to flow in Pulmonary Artery is uncommon.

Clinical presentation
Decreased PBF
Cyanosis
SpO2

Increased PBF
Mild
85 90 %

Moderate to severe
65 70 %

Symptoms

Resting hyperventilation, Dyspnoea , tachypnoea, cyanotic spells , Poor feeding, altered sensorium, Excessive perspiration metabolic acidosis

Physical examination
Decreased PBF Increased PBF
Pulses Apex Cardiomegaly Decreased LV Absent Normal except with coarctation LV, Hyperdynamic Present

S1
S2 S3

Single , loud
Single , loud Absent

Single , loud
Split , loud P2 Present

Physical examination
Decreased PBF Increased PBF
Murmur 2-4/6 systolic murmur at left lower lateral sternal border Or ESM in pulmonary area Clear Loud, harsh,4-5/6 PSM at left lower sternal border, MDM at apex Crepitations

Lungs

Liver

Normal or just palpable

Palpable 4 6 cm below costal margin

Complications

Cerebral embolism Cerebral abscess Infective endocarditis Progressive LV dysfunction Pulmonary hypertension Impaired growth

Differential diagnosis

Infant with cyanosis

Pulmonary atresia with intact septum, Severe PS with VSD Single ventricle with severe PS TGA , VSD with PS DORV, VSD with PS

Infant with failure

TGA DORV without PS Taussing Bing type Single ventricle without PS TAPVC Common Atrium Persistent truncus arteriosus

Electrocardiograph

Chest radiograph

TA with VSD without PS

Chest radiograph

TA with PS

Tricuspid atresia with TGA

Echocardiography

Important parameters :
Size of atrial communication Size of RA & RV Presence / severity of PS Presence / size of VSD Presence / size of PDA Relationship of Aorta/PA Degree of MR LV function

Cardiac Catheterization
Indications :

To determine the hemodynamics, measure pulmonary artery pressure and to estimate pulmonary vascular resistance following palliative surgical intervention prior to next final operation.

To define sources of pulmonary blood flow and associated cardiac anomalies not clearly defined by echocardiography. ( can be seen with MRI/CT ) For balloon septostomy in some cases with restrictive interatrial communication (Can be done under echocardiography guidance )

Cardiac Catheterization
PBF
PBF

Angiocardiography

Rarely needed nowadays with present day non-invasuve modalities except in cases where exact PA pressure and pulmonary vascular resistance are necessary.
To evaluate pulmonary artery anatomy, define associated anomalies & visualization of collaterals , other modalities which can be used are - MRI ( preferred since no radiation exposure ) - CT

Treatment
The goals of treatment in the newborn infant is to

(a) provide pulmonary blood flow adequate to avoid extreme hypoxemia;

(b) prevent pulmonary overcirculation and pulmonary hypertension, which can lead to left ventricular failure or pulmonary vascular disease; and (c) preserve pulmonary artery anatomy for later surgery.

Role of PGE1

Indication : severe restriction to pulmonary blood flow is present at birth

Mechanism of action : It maintains patency of the ductus arteriosus and ensures adequate pulmonary blood flow until surgery can be performed. Dose : an infusion of PGE1 (0.025 to 0.1 mg/kg per minute) Precautions : Intubation and mechanical ventilation may become necessary because of the potential apnoea caused by prostaglandin in a small percentage of patients. Other side effects for which these patients must be monitored include seizures, fever, and hypotension resulting from peripheral vasodilation, necrotising enteritis.

Treatment of cyanotic spell

Knee chest position

Oxygen supplementation
Morphine 0.1 mg/kg intravenous or subcutaneous

Volume expansion
NaHCO3 1 meq / kg Alpha agonist phenylephrine 5 10 mcg/kg/min Propranolol 0.1 0.2 mg / kg over 5 min

Tricuspid atresia treatment protocol

Initial palliative procedure

Ia & IIb (decreased PBF) : Aortopulmonary shunt

Ic ( Increased PBF) : Pulmonary artery banding to prevent overcirculation and to eliminate pulmonary hypertension and possible pulmonary arteriolar damage.

Later : Fontan pathway

Glenn shunt : 5 mth 1 year Fontan completion : 2 3 years

Tricuspid atresia with TGA

Initial palliative procedure

IIa and IIb (decreased PBF) : Try to enlarge the VSD directly or create an aortopulmonary shunt ( MPA is anastomosed to the ascending aorta - Damus-Kay-Stansel procedure).

IIc ( Increased PBF) : Pulmonary artery banding to prevent pulmonary overcirculation and to eliminate pulmonary hypertension and possible pulmonary arteriolar damage

Later : Fontan pathway

Shunts

Modified Blalock Thomas - Taussig shunt : Subclavian artery to the ipsilateral pulmonary artery anastomosis using prosthetic material (e.g., Gore-Tex tube) , the procedure of choice nowadays.

Blalock -Thomas- Taussig shunt : subclavian artery to the ipsilateral pulmonary artery
Potts shunt : descending aorta to distal left pulmonary artery Watersons shunt : ascending aorta to proximal right pulmonary artery

Modified BT shunt
Indications :

Sao2 < 70 % Cyanotic spells If Glenn shunt cannot be done.

Bidirectional Glenn shunt

Superior vena cava to Right Pulmonary artery . Indications :

Shunt inadequate/failure.
Elective by 5 mth - one year of life.

Indications for Fontan procedure

Recurrence of the cyanosis Progressive polycythemia Decreasing exercise tolerance Shunt failure or Increasing pulmonary obstruction Elective by 2 - 3 years of life.

Criteria for optimal results following the Fontan procedure

Age at operation between 4 and 15 years The presence of normal sinus rhythm Normal systemic venous connections Normal right atrial size Normal pulmonary arterial pressure (mean <15 mm Hg) Low pulmonary vascular resistance (<4 Woods units/m2) Adequate-sized pulmonary arteries with diameter >75% of the aortic diameter Normal left ventricular ejection fraction (60%) An absence of mitral valve insufficiency Absence of complicating factors from previous surgeries, such as pulmonary artery distortion

Fontan procedure

First described by Francis Fontan in 1968. Has undergone many modifications over the years.

Lateral tunnel Fontan

Extracardiac Fontan

Complications of Fontan procedure

Persistent pleural effusion Atrial arrhythmias Thromboembolism Protein losing enteropathy LV dysfunction Development of venous and arterial collaterals.

Prognosis

1-year survival rate without surgical palliation as low as 10%, depending on the type of tricuspid atresia and associated lesions . The overall perioperative mortality rate of patients undergoing the modified Fontan procedure in recent series is < 5%. Nowadays patients undergoing a modified Fontan procedure have excellent outcomes.

10-Year Survival After Fontan-Type Operation

Author
Fontan et al

Year
1990

No of pts
334

Years of surgery
1975-1988

survival
69 %

Driscoll et al
Cetta et al Gentles et al Weipert at al Giannico et al

1992
1992 1997 2004 2005

352
339 500 162 221

1973-1984
1987-1992 1973-1991 1978-1995 1988-2003

70 %
81 % 79 % 83 % 85 %

Survival after surgery

A cross-sectional analysis of 256 patients undergoing a modified Fontan after Glenn shunt between 1994 and 2007. Results: Survival was 97% , 96% %, and 94% , respectively, at 1, 5, and 10 years. Event-free survival was 96%, 87% %, and 64%, respectively, at 1, 5, and 10 years .

Conclusions: Survival for patients undergoing a completion Fontan in the current era is excellent, but patients remain at risk for morbid events.

Ann Thorac Surg 2009;88:1291-1299.

Thank you