Pediatric Tricuspid Atresia Follow-up
- Author: P Syamasundar Rao, MD; Chief Editor: Howard S Weber, MD, FSCAI more...
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]
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. 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.
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.
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.
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, calcium replacement, regular high-molecular-weight heparin and low-molecular-weight heparin,[148, 149, 150] somatostatin, high-dose spironolactone, sildenafil, and resection of localized intestinal lymphangiectasia (if demonstrated) 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.
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.
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.
Inpatient & Outpatient Medications
See the list below:
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.
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.
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.
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.
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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