Pulmonary Atresia With Intact Ventricular Septum Treatment & Management

Updated: Dec 27, 2020
  • Author: John R Charpie, MD, PhD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Treatment

Approach Considerations

Early decision making on the management goal is crucial, as determined by individual morphologic characteristics at presentation, and outcomes may be improved by right ventricular development at a later time point is taken into consideration. [10] Note the following:

  • Consult with a pediatric cardiologist and pediatric cardiothoracic surgeon for the management of patients with pulmonary atresia with intact ventricular septum (PAIVS).

  • Admit patients with PAIVS for future preoperative testing and surgical interventions. Note that pulmonary valve replacement constitutes most late reoperations. Transfer is required for specialized diagnostic evaluation and surgical intervention.

  • Possible discharge medications include digoxin, furosemide, and aspirin. Outpatient management requires carefully monitoring of medication doses and adverse effects. In addition, monitor the adequacy of repair/palliation with pulse oximetry and periodic echocardiography.

  • Patients with PAIVS require increased caloric density during infancy to provide 120-130 kcal/kg/d for approximately 6 months.

  • No specific activity restrictions are necessary. [11]

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Medical Care

Initial treatment of pulmonary atresia with intact ventricular septum (PAIVS) consists of maintaining ductal patency with a continuous intravenous prostaglandin E1 infusion.

To correct metabolic acidosis in a neonate, replace fluids and administer sodium bicarbonate. Mechanical ventilation may be necessary if acidosis persists and apnea occurs secondary to prostaglandin E1.

Patients ultimately require surgical palliation or therapeutic catheterization prior to hospital discharge.

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Surgical Care

An optimal strategy for definitive repair has yet to be defined. [2] Surgical or catheter-based interventional algorithms for pulmonary atresia with intact ventricular septum (PAIVS) depend on the size and morphology of both the tricuspid valve and the right ventricle, as well as the presence of abnormal coronary artery anatomy.

In a retrospective study, Rathgeber et al reviewed all cases of PAIVS treated with radiofrequency perforation (RFP) at a single center from 1999 through 2012 (n = 18) to determine the safety and efficacy of RFP and to assess the pre-intervention anatomical parameters associated with a biventricular outcome. Seventeen procedures were successful; all patients were alive at the most recent follow-up (median: 4.9 years), 12 (71%) had a biventricular circulation, 2 (12%) had a 1½ ventricle repair, 2 (12%) had a univentricular repair, and 1 was lost to follow-up. In the investigators’ cohort of patients, the pre-intervention tricuspid valve/mitral valve (TV/MV) ratio and tricuspid valve (TV) z-score were found to be predictors of a biventricular outcome. [12]

In a retrospective study, Zheng et al reviewed their 15-year experience in the management of PAIVS. The patients (n = 170) were divided into two groups: the one-stage surgery group (n = 33) and the staged surgery group (n = 137), in which patients received definitive repair, including biventricular repair, 1.5 ventricular repair and univentricular palliation without or with initial intervention. In the one-stage surgery group, 3 patients died postoperation; the estimated 1-, 5- and 15-year survival rates were 97.0%, 93.7%, and 88.5%, respectively. In the staged surgery group, 23 patients died; the estimated 1-, 5- and 15-year survival rates were 89.8%, 88.2%, and 69.1%, showing no significant difference from the one-stage surgery group. [13]

Invasive fetal cardiac intervention is a novel approach to the management of PAIVS. Fetal pulmonary valvuloplasty has been performed at several institutions with varying degrees of success. [14, 15] Hogan et al reviewed International Fetal Cardiac Intervention Registry data to evaluate the fetal and maternal characteristics, the technical aspects of the procedure, as well as pregnancy and neonatal outcome data. [14] The procedure was considered successful in 41 of 58 (71%) cases. Nine fetuses died (7 periprocedure and two >48 hrs postprocedure). The authors noted extreme variation among center criteria for the procedure and recommend prospective case-controlled studies using uniform criteria to increase the likelihood of successful outcomes. [14]

Mild tricuspid valve and right ventricular hypoplasia without ventriculocoronary connections

Perform transcatheter valvotomy, with or without stenting of the patent ductus arteriosus or surgical systemic-to-pulmonary artery shunt placement.

In 10 neonates with PAIVS, Li and colleagues showed that pulmonary valve perforation and balloon valvuloplasty through the right ventricle was a safe and feasible alternative to surgical valvotomy. [16] If the right ventricle and tricuspid valve grow, a two-ventricle correction is probable in the future.

One-stage definitive repair has been described in two infants. [17] The repair comprised resection of hypertrophied muscles in the outflow and trabecular portions of the right ventricle (right ventricular overhaul technique), surgical valvotomy or transannular patch, and adjustable snare-closure of the foramen ovale.

A study by Chubb et al summarized long-term followup of 39 patients with PA/IVS who underwent valve perforations with or without stenting of the arterial duct. There were 8 deaths (21%), and 25 patients (83% of survivors) had a biventricular circulation despite small median tricuspid valve Z-scores. [18]

Hasan and colleagues [19] reviewed the short- and medium-term outcome of transcatheter pulmonary valve perforation in 50 patients with PAIVS and non–right ventricular–dependent coronary circulation. Pulmonary valve perforation was attempted in 30 patients, and 26 had a successful procedure. Twenty-four patients had surgery without pulmonary valve perforation. Complications of pulmonary valve perforation included myocardial perforation in 17% of patients. There were no deaths in the entire cohort. Tricuspid valve z-scores were larger in the nonsurgical patients, and all patients in the nonsurgical group achieved a biventricular circulation at a median follow-up of 4.3 years.

Moderate-to-severe tricuspid valve and right ventricular hypoplasia without ventriculocoronary connections

Perform a surgical valvotomy or transannular patch with a systemic-to-pulmonary artery shunt or a transcatheter valvotomy with stenting of the patent ductus arteriosus. Future 1.5-ventricle or univentricular (Fontan) repair is likely.

Moderate-to-severe tricuspid valve and right ventricular hypoplasia with ventriculocoronary connections but no stenoses or interruption

Perform a surgical valvotomy or transannular patch with a systemic-to-pulmonary artery shunt or a transcatheter valvotomy with stenting of the patent ductus arteriosus. Future 1.5-ventricle or univentricular (Fontan) repair is likely.

Moderate-to-severe tricuspid valve and right ventricular hypoplasia with ventriculocoronary connections and proximal stenoses or interruption

Perform a systemic-to-pulmonary artery shunt or stenting of the patent ductus arteriosus. Future univentricular (Fontan) repair or heart transplant is likely.

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