Surgical Treatment of Double Outlet Right Ventricle Complicated by Pulmonary Hypertension

Abstract

Background:Double outlet right ventricle (DORV) is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension (PH) is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH.Methods:From June 2004 to November 2016, 61 patients (36 males and 25 females) aged 2 weeks to 26 years (median: 0.67 years and interquartile range: 0.42–1.67 years) with DORV (two great arteries overriding at least 50%) complicated by PH underwent surgical treatment in our center. All patients were categorized according to surgical age and lesion type, respectively. Pulmonary artery systolic pressure (PASP), pulmonary artery diastolic pressure (PADP), and mean pulmonary artery pressure (mPAP) were measured directly before cardiopulmonary bypass (CPB) was established and after CPB was removed. An intracardiac channel procedure was performed in 37 patients, arterial switch procedure in 19 patients, Rastelli procedure in three patient, Senning procedure in one patients, and Mustard procedure in one patient. The Student's t-test and Chi-squared test were performed to evaluate clinical outcomes of the surgical timing and operation choice.Results:Fifty-five patients had uneventful recovery. PASP fell from 55.3 ± 11.2 mmHg to 34.7 ± 11.6 mmHg (t = 14.05, P < 0.001), PADP fell from 29.7 ± 12.5 mmHg to 18.6 ± 7.9 mmHg (t = 7.39, P < 0.001), and mPAP fell from 40.3 ± 10.6 mmHg to 25.7 ± 8.3 mmHg (t = 11.85, P < 0.001). Six (9.8%) patients died owing to complications including low cardiac output syndrome in two patients, respiratory failure in two, pulmonary hemorrhage in one, and sudden death in one patient. Pulmonary artery pressure (PAP) dropped significantly in infant and child patients. Mortality of both infants (13.9%) and adults (33.3%) was high.Conclusions:PAP of patients with DORV complicated by PH can be expected to fall significantly after surgery. An arterial switch procedure can achieve excellent results in patients with transposition of the great arteries type. Higher incidence of complications may occur in patients with ventricular septal defect (VSD) type before 1 year of age. For those with remote VSD type, VSD enlargement and right ventricle outflow tract reconstruction are usually required with acceptable results. The degree of aortic overriding does not influence surgical outcome.

Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease

“…However, PAH-CHD with 6 WU/m 2 < PVRI < 10 WU/m 2 or 0.3 < PVR/SVR < 0.5 is a “gray area” because there has been no systematic study or high-quality evidence that can be used to predict a prognosis, [4,22] so the decision to operate and the therapeutic policies for severe PAH-CHD in children aged above 2 years remain challenging and the prognosis for surgical intervention is unpredictable. [23]…”

A case of complex congenital heart malformation in an adult: double Inlet-Double Outlet Right Ventricle

“…Most of the previous points have been reported in individual cases. Preoperative pulmonary hypertension (PH) is considered to be an essential risk of early death during double outlet Right Ventricle (DORV) surgery [5] , with overall mortality or heart transplant rate of 36% [1] . Accurate diagnosis using non-invasive imaging techniques is essential for optimal treatment [1] .…”

Repair of double outlet right ventricle: Midterm outcomes

“…Double outlet right ventricle (DORV) is a complex cardiac malformation with ventriculoarterial connection in which both pulmonary artery and aorta arising primarily from the right ventricle (RV), typically associated with discontinuity between the aorta and mitral valve. [1][2][3][4][5] The surgical management of DORV ranges from single ventricle palliation (SVP) to biventricular (BiV) repair. The BiV approach includes either primary complete repair, or staged repair in which intracardiac repair is distributed over a series of operations.…”