Tricuspid Atresia Follow-up

Updated: Sep 25, 2016
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Richard A Lange, MD, MBA  more...
  • Print
Follow-up

Further Outpatient Care

For patients who have undergone a palliative procedure, arrange careful follow-up to monitor pulmonary blood flow. Increasing polycythemia and cyanosis are indicative of poor shunt flow and may indicate that another procedure is needed to prevent worsening hypoxemia.

Increased pulmonary blood flow is detrimental in this population; monitor carefully, watching for signs of congestive heart failure.

For patients who have had the Fontan procedure, maintain follow-up care to ensure a stable cardiac rhythm. Consider careful assessment of ventricular function on a routine basis. These patients may develop signs of congestive heart failure and congestive hepatopathy in the early postoperative course. In such instances, institute diuretic therapy early in order to preserve pulmonary blood flow and oxygenation.

Next:

Further Inpatient Care

Because pulmonary blood flow is of paramount importance in patients with tricuspid atresia, carefully monitor for signs of hypoxemia or fluid overload. After a palliative procedure, perform echocardiographic assessments to determine the patency of the shunt. Clinical evaluation is of benefit to determine instances of increased pulmonary blood flow that require treatment for ensuing congestive heart failure. Signs of hypoxemia in the form of rising hemoglobin levels must be monitored carefully because onset is insidious.

Inpatient care after the Fontan procedure requires careful monitoring of pulmonary vascular resistance, heart rhythm, and fluid status. All efforts are made to maintain pulmonary vascular resistance as low as possible using supplemental oxygen and pulmonary vasodilators. Maintaining normal sinus rhythm in these patients optimizes cardiac output and ensures a favorable outcome. These patients characteristically develop pleural effusions after the procedure, which must be monitored carefully and removed in order to maximize oxygenation and decrease pulmonary vascular resistance.

Carefully examine the child for signs of hepatic congestion. Hepatic congestion can occur secondary to the operative procedure and the subsequent volume overload. Treatment consists of diuretic therapy.

Previous
Next:

Inpatient & Outpatient Medications

Consider digitalis to maintain sinus rhythm and improve cardiac contractility.

Diuretics are often administered to treat and prevent pulmonary edema in these patients.

Previous
Next:

Complications

The complications from a shunt procedure can include too much pulmonary blood flow. The shunt can also cause damage to the pulmonary arteriolar tree.

Complications from the Fontan procedure include pulmonary edema, congestive hepatopathy, pleural effusions, ascites, protein-losing enteropathy, and cardiac arrhythmias.

Previous
Next:

Prognosis

The 1-year survival rate after the Fontan operation is 85%; the 5-year survival rate is 78%. Because the procedure eliminates cyanosis, polycythemia and left ventricular volume overload are relieved; therefore, this population can be expected to live longer.

Previous
Next:

Patient Education

Counsel the parents of these children about dietary sodium restriction, as well as the signs and symptoms of hypoxemia and congestive heart failure.

Previous