J Tisnado, MD, FACR, FACC, FSIR, FAHA1 J Tisnado2, D J Komorowski1, M K Sydnor1

1MCV

Hospitals/VCU Medical Center, Richmond VA 2Hartford Hospital, Hartford CT

Central catheterization, a well-established procedure managing clinical entities, such as: hemodialysis, total parenteral nutrition, IV access, blood draws, and apheresis, among others. Procedures are simple and straight forward in patients with normal or conventional cardiac and central venous anatomy. We place central catheters in patients with different and varied congenital cardiac and venous vascular abnormalities, both congenital and acquired. A review of our experience with central catheterization in these circumstances is in order. We briefly discuss some interesting instances of cardiovascular problems, and provide guides for successful insertions. Patients with different cardiac anomalies, i.e. transposition of great vessels, double SVC, left-sided SVC, Tetralogy of Fallot, post-Fontan and post-mustard procedures and others. Many patients with left ventricular assist devices, aortic balloon pumps, and artificial hearts are also illustrated in this educational exhibit.

Successful placements can be achieved in most, if not all, patients with different cardiac and central venous vascular anomalies if one is aware of the vascular anatomy and cardiac malformation and base some information about the different operations to correct congenital heart disease. Placement of central catheters in patients with cardiac and central venous vascular anomalies is relatively easy and safe, especially if one has a basic knowledge of the anatomical configuration prior to placement. Noninvasive imaging: US, CTA, MRA are the main pre-procedure imaging methods to plan the approach. Digital subtraction venography is necessary in complicated and confusing cases. We present a brief review with actual examples and describe some of the tricks-of-the-trade to be successful in the placement of central catheter in these uncommon situations. Percutaneous translumbar placement of central venous catheters can be done also without any differences in complication rates than the "routine" placements of central catheter placement. The translumbar placement is safe and reliable in children.

In general, children with congenital heart disease need long-term indwelling catheters for a long time for a long-term administration of IV fluids and drugs, samplings of blood and/or TPN. Sometimes the central veins are no longer available for us. Sometimes the patients need preservation of the SVC or IVC for future interventions, surgery and/or catheterizations. Central venous catheterization is nowadays "routine" management in the armamentarium of an aggressive health care team.
A) percutaneous non-tunneled catheter placement in the SVC, IJV, External jugular veins (EJV), femoral veins (FV) B) peripherally inserted central catheters (PICCs) C) percutaneous tunneled catheters valved: Groshong non-valved: Hickman, Broviac D) implantable ports

If the access is difficult or depleted or needs to be preserved, alternative approaches are needed. The translumbar approach to the IVC and right atrium may be the ideal in some aspects.

In general, 9 out of 1000 (1%) babies are born with a congenital heart abnormality. In order to prevent the death of those babies, 2.3 of those babies need some type of treatment of the congenital heart disease. Further, 1 million of people in USA were born with some type of congenital heart defects. Furthermore,  15% of children 12-17 are smokers already;  1/2 of children 12-21 do not exercise daily;  30% of children in USA are obese. About 3.5-5 million of central venous catheters are placed annually in USA -- 250,000 CVC related infections in this group. The Fontan procedure is initially a connection between the right atrium to the pulmonary arteries. Variations: A cavopulmonary connection with a tunnel either intracardiac or extracardiac. Placement of an external conduit bypassing the right atrium completely. Central venous catheter placement is preferred from the IJV in to the brachiocephalic veins only. No attempts to place catheters into the SVC should be made.

VCU is one of the leading institutions in the country in placing TAH. Usually the TAH is powered by a large, 400 lbs console. Hopefully, in the near future, it will be powered by a 13-pound backpack so the patients can go home to wait for a heart. TAH is indicated as a bridge to a human heart transplant, in patients dying from end-stage biventricular failure.

Average waiting time for a heart is about 3 months for patients with artificial heart. Since the console to drive the TAH is a large one, 400 lbs., the patients must stay in the hospital awaiting for an organ. With the new back pack power, the patients could go home to await for a heart. SynCardia: Total temporary artificial heart. Therefore, in the future, we may see outpatients with TAH and we must be prepared to insert central venous catheters in those patients. Furthermore, it is very important to know the anatomy of those patients before insertion of central catheters. Andropoulos et al reported 450 central venous catheter placements in children with congenital heart disease. They recommended that the tip of the central catheter be placed in the SVC, just above the right atrium. They presented an interesting formula to predict the placement of the tip of the catheter and to prevent serious perforations of the heart or great vessels. Other complications mentioned are: dysrhythmia, extravasation of IV fluids, thrombosis, inaccurate measurements of central venous pressures and tamponade due to cardiac perforation. They recommend to place the central venous catheters with the patients in Trendelemburg and using anatomic landmarks or palpation of the carotid arteries or with Doppler US guidance.

This is routine for us as we always use US to access the central veins. We assume that those guidelines are for those operators inserting central venous catheters with no imaging guidance which is not recommended by us, especially when one is dealing with children with congenital heart disease. Qureshi et al advocate a transhepatic placement of central catheters to avoid or prevent central venous thrombosis and also to preserve the central veins for future use, specially in children with complex congenital heart diseases. In their experience 32 children had 40 transhepatic placements. A complication rate of 8.8% was reported. We have not done this since we believe that the transhepatic approach is too aggressive and drastic and we reserve these optional approaches such a transhepatic and translumbar for patients with depleted central veins. An interesting study comparing thrombotic complications between heparincoated central catheters vs. non-heparin-coated catheters was reported recently. No advantage of one over the other was demonstrated in infants with congenital heart diseases. Both exhibited similar rates of complications (42% vs 44%).

Some patients with patent foramen ovale (PFO) who need central venous catheters must have continued treatment with "blood thinning" drugs such as clopidogril , aspirin or Coumadin. These patients have a high rate (40%) of stroke. In addition these patients must have "endocarditis prevention" with antibiotics, specially if they are having dental work, any "invasive" test, or minor or mayor surgery. Otherwise, the methods of placement of central catheters are the normal ones, i.e. the tip of the catheter in the right atrium-SVC junction or in the right atrium. Regarding PICCs they must be placed always under fluoroscopic guidance. A study of 843 PICCs placed in 698 children by the "PICC" team or operators without fluoroscopy showed that 86% of central catheters were misplaced and needed repositioning under fluoroscopy to achieve a 90% success of correct central venous catheter placement. Conclusion of the study: PICCs must be placed under fluoroscopy. This applies to children with and without congenital heart disease. By the way. The tips of the PICC were considered as central if placed anywhere within the SVC. Superior border limited by the right tracheobronchial angle. The inferior border defined at slightly below the right lateral border of the heart in the chest X ray.

VADs are used to assist the failing heart, usually temporarily after a MI or permanently in patients with end-stage heart failure. VAD aid the heart in maintaining the ventricular output. Initially, and still are used temporarily to help the ventricle to recover or as a bridge for heart transplantation. Sometimes the VAD are permanent is some patients who are not candidates for heart transplant and whose heart will not recuperate. In general the indications for a VAD are:
a) temporary to recovery of the heart (days and weeks). b) temporary, as a bridge for heart transplantation. Patients are awaiting for an organ. c) permanent for patients no candidates for transplant. Use until death.

The device is normally inserted in the thorax and abdomen. The inflow cannula is connected into the tip of the ventricle and the outflow cannula returns the blood to the ascending aorta or main pulmonary artery. The pump is usually located in the abdomen either intra- or extra-peritoneal. In general there are two types of VAD.
a) continuous flow b) pulsatile flow

Regardless of the type of VAD, the patients may have, the placement of central catheters is rather straightforward. The access can be the IJV or the EJV or the SCV for central catheters and the arm veins (basilic) for PICCs. The tip of the catheter is placed usually in the SVC-right atrial junction or preferable in the middle of the right atrium as we prefer.

Placement of central catheters in patients with patent foramen ovale (PFO) is rather straightforward. However, care must be taken to make sure no clot or foreign body (fibrin deposits) form around the catheter. Otherwise, serious paradoxical emboli may occur. A small clot could embolize and result in a stroke if enter the cerebral arteries or a MI if enter the coronary arteries. There have been studies demonstrating that heparincoated-bonded central venous catheters do not reduce thrombosis incidence, at least in infants less than one year-old with congenital heart diseases. Therefore, special care is needed in these infants since they usually require for a much longer time the central venous catheters.

The total artificial heart (TAH) is a temporary device for patients who have lost the function of both ventricles and, therefore, cannot have a VAD. The TAH allows the recovery of patients for them to be in better shape for a heart transplant be done in about 2-3 months ideally. More than 20 patients have been done at our institution. The patients must remain in the hospital as the console to drive the unit is huge. Portable devices are being developed now. In these patients care must be taken during placement of the central catheters as a too long catheter into the right atrium could result in prolapse of the catheter into the artificial heart creating problems for functioning. Ideally the central venous catheters in these patients should be in the SVC-right atrial junction. Placement of the tip in the right atrium is also acceptable.

Two infants with congenital heart disease had thrombosis of the veins after placement of a central catheter in the L IJV. One woman with a persistent L SVC died due to extensive thrombosis of the coronary sinus after placement of a central venous catheter in the L SVC. Another infant with a total anomalous venous return had placement of a central venous catheter in the L IJV which drained into a persistent L SVC, which became occluded after central venous catheter placement with a resultant left upper lobe consolidation due to obstruction of the venous outflow of the left upper lobe. Therefore, a complete evaluation of the venous anatomy, the systemic and the pulmonary venous anatomy is important before inserting central venous catheters in children with congenital heart disease, particularly in cases of total or partial anomalous venous return. Perhaps in these infants, placement of central catheters in the right side veins, such as R IJV, R SCV, R EJV, is preferred or else the central veins should be avoided altogether and the central catheters inserted into the IVC and other alternate places like transhepatic, translumbar.

AC is a localized "diaphragm-like" stenosis of the thoracic aorta occurring usually just distally to the origin of the L SC artery. In addition there is usually a bicuspid aortic valve. Most patients are asymptomatic due to development of collaterals. In these patients the placement of central venous catheters is rather simple and straightforward as in the "normal patients.

Total pulmonary venous return occurs when all the pulmonary veins return to the right atrium directly or indirectly via the SVC or IVC. Partial anomalous venous return occurs when not all the pulmonary veins return to the systemic circulation such as to the right atrium, SVC or IVC. In these patients it is better not to place central venous catheters in the neck or chest, i.e. into the central veins, since thrombotic complications of central venous catheterization could result in serious and even lethal "paradoxical" emboli. It is better to place catheters in the IVC or in the femoral veins.

The ductus arteriosus in the fetal stage usually connects the left pulmonary artery with the proximal descending aorta, distally to the origin of the L SC artery. During fetal life the blood form the left pulmonary artery bypasses the lungs directly into the descending aorta. In patients with PDA (left to right shunt) the placement of central venous catheters should be straightforward. Unless there has been a reversal of flow (right to left shunt, Erlenmeyer complex). In these cases care must be taken to prevent thrombi forming in the central catheter. A small thrombus associated with the central catheter could be very serious, indeed.

Are communications between the atria. ASD are the most common congenital heart diseases in adults, at least. There are 3 types of ASD:
1. 2. 3.

Ostium primum, Ostium secundum, and sinus venosus.

Are the most common congenital heart diseases in children. The VSD can be located in the membranous or in the muscular septum or above the atrioventricular septum or near the mitral and tricuspid valves. The placement of central catheters in patients with ASD or VSD should be straightforward too. However, in adults with large VSD with enlargement and hypertrophy of the right ventricle and pulmonary hypertension, special care must be taken to make sure no thrombi form in the central venous catheter. Otherwise, serious paradoxical emboli could occur.

Consists of four anomalies:

1. 2. 3. 4.

subpulmonary infundibular stenosis, VSD, right ventricular hypertrophy, and overriding aorta.

Patients with T of F usually are operated early in life; therefore, the placement of central catheters can be done with no special or particular problems or precautions.

In these patients a single vessel (trunk) originates from the heart. The patients are usually operated early in life. The placement of central catheters in these patients is done by puncture of the IJVs or the EJVs in either side. Ideally, short catheters with the tip in the innominate veins or the SVC should be used.

Is one of the most common congenital heart diseases. In these patients the aorta originates from the right ventricle and the pulmonary artery originates from the left ventricle. Most patients have no other cardiac anomalies. A left to right shunt is necessary for life. Since the great vessels are reversed, one can place central venous catheters in the IJVs or EJVs or the SCV or SVC. It is better not to place catheters in the atria as thrombi can develop and embolize with resultant rather serious complications.

ASD comprises about 19% of all congenital heart diseases. There is a risk of paradoxical emboli. VSD is one of the most common congenital heart diseases. There are 4 types, depending on where in the septum is the defect. PDA is a communication between the left pulmonary artery and the descending aorta which usually obliterates within 2-3 weeks of life. It represents about 5-10% of all congenital heart diseases.

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