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Pediatric Tricuspid Atresia Follow-up

  • Author: P Syamasundar Rao, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Jan 05, 2016
 

Further Outpatient Care

Periodic follow-up after surgical correction is necessary. Some children need continued support with inotropes and/or diuretics. Angiotensin-converting enzyme inhibitors to effect afterload reduction are generally prescribed to augment left ventricular output with consequent improvement of forward pulmonary flow. Although this concept has theoretical advantages, no data from control studies are available to substantiate this thesis.

Anticoagulation with warfarin is recommended because of risk of thrombi development in the right atrium in the conventional Fontan procedure. Whether such recommendation is valid for total cavopulmonary connection is not clear. As an alternative, platelet-inhibiting doses of aspirin 5-10 mg/kg/d in children or clopidogrel 75 mg/d particularly in adults may be used,.

Post-Fontan surgery complications

Whereas most patients do well after Fontan surgery, some develop problems, including arrhythmia, obstruction at the anastomotic sites or in the pulmonary artery, residual shunts, and/or systemic venous congestion (eg, protein-losing enteropathy).[130, 131, 132, 133, 134, 135]

Arrhythmias

Atrial flutter and/or fibrillation and supraventricular tachycardia should be treated with appropriate pharmacologic therapy. If arrhythmias are not adequately controlled, electrophysiologic study and transcatheter or surgical ablation (Maze procedure) may be indicated. Revision of the classic Fontan procedure to total cavopulmonary connection with elimination of the enlarged right atrium should also be considered; some reports indicate success with this approach.[136] Atrioventricular block and sick sinus syndrome may be observed in some children; in such patients, pacemaker insertion may be necessary. Ventricular arrhythmias are infrequent.

Obstructed Fontan pathways

Any signs and symptoms suggestive of obstruction in the Fontan circuit should be promptly investigated. Poor echo windows may make this evaluation difficult. MRI and magnetic resonance angiography (MRA) may be helpful in defining these obstructions. Cardiac catheterization and angiography may be needed to confirm or exclude such obstruction. If notable obstruction is present, balloon angioplasty, stent placement, and, if necessary, surgery should be undertaken to provide prompt relief.[40]

Residual shunts

Residual atrial defects and/or intentional Fontan fenestration may cause substantial arterial hypoxemia. Cerebrovascular accidents and systemic emboli are reported, presumably due to paradoxical embolism. In these situations, transcatheter occlusion of the fenestration is recommended. Test occlusion of the defect should be performed to ensure adequate cardiac output. Most such defects are amenable to transcatheter occlusion. Regular interatrial fenestrations and residual atrial septal defects (ASDs) can be transcatheter occluded by the conventional atrial septal closing devices. Tubular fenestrations such as those associated with an extracardiac Fontan operation may require coils or other devices (eg, Amplatzer duct occluder or Amplatzer vascular plug; AGA Medical, Golden Valley, Minnesota).[87, 92, 93]

Residual systemic to pulmonary or systemic venous collaterals

Also search for residual systemic to pulmonary or systemic venous collaterals that might result in additional left ventricular volume load or residual right-to-left shunting, respectively. These are also treatable with coil or vascular plug occlusion. Late follow-up results after fenestration closure appear encouraging.[89]

Chronic systemic venous congestion may occur.

Protein-losing enteropathy, recurrent pleural effusion, and liver dysfunction are noted in a small percentage of patients after Fontan surgery.[137, 138, 139] Protein-losing enteropathy results in a high mortality rate. Its etiology is unknown.

Although protein-losing enteropathy has been reported in total cavo-pulmonary connection type of Fontan, prevalence in the total cavo-pulmonary group may be lower (1.2%) than that seen in atriopulmonary connection type (11.1%) of Fontan.[139, 140, 141]

Loss of protein in the bowel appears to be secondary to lymphatic distension, which may be due to elevated systemic venous pressure. However, this phenomenon is also observed in patients with normal pressures for the Fontan circuit. Symptoms usually manifest 6 months or later after a Fontan procedure. Diarrhea, edema, ascites, and/or pleural effusions are the usual findings at presentation. Hypoalbuminemia and elevated alpha-1-antitrypsin levels in the stool are present. Confirmation of the syndrome with technetium 99m-labeled human serum albumin scintigraphy may be useful.[142]

Obstruction in the Fontan circuit must be evaluated for and, if present, relieved by means of transcatheter or surgical therapy. Likewise, aortopulmonary connections[140, 143] and naturally occurring or previous surgical shunts should be sought out and closed with transcatheter methods or surgery. A diet of medium-chain triglycerides and the administration of parental albumin are supportive. Replacement of immunoglobulins may also be considered. Prednisone,[144, 145] an elementary diet,[146] calcium replacement,[147] regular high-molecular-weight heparin and low-molecular-weight heparin,[148, 149, 150] somatostatin, high-dose spironolactone,[151] sildenafil,[152] and resection of localized intestinal lymphangiectasia (if demonstrated)[153] have been used with variable success.

Reduction of right atrial pressure by creating an ASD has been helpful in some patients. Numerous reports show success with this method.[154, 155, 156]

Protein-losing enteropathy is a potentially fatal complication and should be aggressively treated. In patients with the atriopulmonary type of Fontan, conversion to total cavopulmonary connection may be helpful,[56, 157] although such converting operations are likely to have high mortality rates. Cardiac transplantation should also be considered. Numerous centers have reported improvement after transplantation.[158]

In patients with so called "failed Fontan," after excluding and addressing obstructions and residual shunts apart from other conventional treatment, consideration for right atrial and left ventricular (atrioventricular sequential) pacing,[159, 160] conversion of atrioventricular Fontan to total cavopulmonary anastomosis,[56, 136] and/or cardiac transplantation[161, 162] should be considered.

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Further Inpatient Care

Most follow-up care of tricuspid atresia is outpatient-based (see Further Outpatient Care). Inpatient care may become necessary to treat postoperative complications.

Hospitalization and inpatient care may be required for medical, transcatheter, or surgical treatment of arrhythmias; for relief of obstructed Fontan pathways by means of transcatheter or surgical methods; for transcatheter or surgical closure of residual shunts; and/or for treatment of issues related to protein-losing enteropathy.

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Inpatient & Outpatient Medications

See the list below:

  • See Medication.
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Complications

Development of bacterial endocarditis, brain abscess, and stroke may be considered as complications of the disease itself. Arrhythmias, obstructed venous pathway, and protein-losing enteropathy are some of the complications observed after Fontan surgery.

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Prognosis

The prognosis of neonates with untreated tricuspid atresia is poor. Early identification, rapid and safe transport to a pediatric cardiology center, noninvasive diagnosis (particularly echocardiography), availability of PGE1 to keep the ductus open, and recent advances in anesthesia and surgical technique have improved the prognosis of these babies.

The second period of mortality during the second decade of life (see the image below) may now be reduced with a timely Fontan operation.

Actuarial survival curves from 3 reported clinical Actuarial survival curves from 3 reported clinical series compiled by Dick and Rosenthal (1992) show a high initial mortality rate in the first year of life, a plateau between the first year and middle of the second decade of life, and a second increase in the mortality rate after the middle of the second decade of life; this second rise is presumably related to impaired left ventricular function. From Dick M and Rosenthal A. The clinical profile of tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. Mt Kisco, NY: Futura Publishing Co; 1982:83, with permission.

Complications associated with the classic Fontan procedure, namely arrhythmia, atrial thrombosis, and perhaps even protein-losing enteropathy, may be decreased with the widespread use of staged cavopulmonary connection. However, the latter impression must be confirmed in long-term follow-up studies.

Although both mortality and morbidity rates appear to have decreased after the classic Fontan circulation was changed to a cavopulmonary connection, long-term follow-up data after the new types of Fontan procedures are inadequate. However, both transcatheter and surgical repeat interventions continued to be needed in these patients.[163]

Mortality and major morbidity issues aside, survivors of Fontan surgery appear to have physical and psychosocial functioning scores lower than those of an American population sample.

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Patient Education

Tricuspid atresia is a complex cardiac defect requiring multiple and sometimes frequent medical, transcatheter, and surgical interventions. A detailed explanation of the cardiac defects (including pictorial drawings and heart models) and treatment required should be given to the parents at the time of diagnosis and repeated as needed.

For patient education resources, see Heart Health Center as well as Tetralogy of Fallot.

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Contributor Information and Disclosures
Author

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

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Cardiac specimen from a patient with the muscular type of tricuspid atresia. The right atrium was opened by cutting through the right atrial appendage (RAA). Note the dimple (arrow) in the floor of the right atrium with muscle fibers radiating around it. An atrial septal defect (ASD) is also shown. From Rao PS, Levy JM, Nikicicz E, Gilbert-Barness EF. Tricuspid atresia: association with persistent truncus arteriosus. Am Heart J 1991, 122:829, with permission.
Systemic arterial saturations. Left ventricular (LV) and aortic (Ao) values are plotted against the pulmonary-to-systemic blood-flow ratio (Qp:Qs). Both type I and type II anatomy are included. Note the curvilinear relationship between the parameters. At low Qp:Qs levels, a slight increase in Qp:Qs produces large increase in systemic oxygen saturation; at high Qp:Qs levels, a further increase does not produce a notable increase in oxygen saturation. The ideal Qp:Qs appears to be 1.5-2.5, which results in oxygen saturations in the low 80s. From Rao PS. Cardiac catheterization in tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co: 1982:153, with permission.
Geographic prevalence of tricuspid atresia by continent. Note that prevalences are similar on all continents except for Australia. This lone exception is thought to be related to the small sample size from Australia. CHD = congenital heart defects; TA = tricuspid atresia. From Rao PS. Demographic features of tricuspid atresia. In: Rao PS, ed, Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:23, with permission.
Actuarial survival curves from 3 reported clinical series compiled by Dick and Rosenthal (1992) show a high initial mortality rate in the first year of life, a plateau between the first year and middle of the second decade of life, and a second increase in the mortality rate after the middle of the second decade of life; this second rise is presumably related to impaired left ventricular function. From Dick M and Rosenthal A. The clinical profile of tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. Mt Kisco, NY: Futura Publishing Co; 1982:83, with permission.
Actuarial survival rate of 100 patients who had tricuspid atresia and who were undergoing a Fontan operation at the Hospital for Sick Children, Toronto, in 1975-1989. The survival rate 5 years after the operation is 70%. From Freedom RM, et al. The Fontan procedure for patients with tricuspid atresia: long-term follow-up. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:377, with permission.
Use of electrocardiographic mean QRS vector (axis) in the frontal plane for the differential diagnosis of a cyanotic newborn with decreased pulmonary blood flow. Associated ventricular hypertrophy patterns and decreased right ventricular (RV) forces are also helpful. From Rao PS. Management of neonate with suspected serious heart disease. King Faisal Spec Hosp Med J 1984 (4):209, with permission.
Frontal plane mean QRS vector in 308 patients, plotted by anatomic type. Most patients with tricuspid atresia type I (normally related great arteries) have an abnormally superior vector, also called left axis deviation. Only one half of patients with tricuspid atresia type II have an abnormally superior vector. Most patients with tricuspid atresia type III (subtype A) have an inferiorly oriented frontal plane vector. From Rao PS, Kulungara RJ, Boineau JP. Electrovectorcardiographic features of tricuspid atresia. In: Rao PS, ed, Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:141, with permission.
Subcostal 4-chamber 2-dimensional echocardiographic view of a neonate with tricuspid atresia shows an enlarged left ventricle (LV), a small right ventricle (RV), and a dense band of echoes where the tricuspid valve echo should be. Atrial and ventricular septal defects and the mitral valve are also seen. Note the attachment of the anterior leaflet of the detectable atrioventricular valve to the left side of the interatrial septum. Reprinted from Rao, Fetal and Neonatal Cardiology, 1990, with permission from Elsevier Science.
Selected cineangiographic frames from superior vena caval (SVC) and right atrial (RA) frontal angiography in 2 patients. Note sequential opacification of the left atrium (LA) and left ventricle (LV) without opacification of the right ventricle. The RA on the right, the LA superiorly, and the LV on the left form a nonopacified right ventricular window (arrow). This is a classic appearance of the muscular variety of tricuspid atresia. From Rao PS. Tricuspid atresia: anatomy, imaging, and natural history. In: Brawnwald E, Freedom RM, eds. Atlas of Heart Disease: Congenital Heart Disease. Vol 12. Philadelphia, PA: Current Medicine; 1997:14.1 with permission.
Selective superior vena caval (SVC) injection for a 4-chamber projection (hepatoclavicular) shows tricuspid atresia and filling of the left atrium (LA) through a somewhat restrictive atrial septal defect (arrows). Note the retrograde filling of the coronary sinus (CS). RA = right atrium. From Schwartz DC, Rao PS. Angiography in tricuspid atresia. In: Rao PS, ed. Tricuspid Atresia. 2nd ed. Mt Kisco, NY: Futura Publishing Co; 1992:223, with permission.
 
 
 
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