Successful management of patients with pulmonary atresia with intact ventricular septum using a three tier grading system for right ventricular hypoplasia.

Abstract

BACKGROUND:

We sought to validate a simple grading scheme for right ventricular hypoplasia in determining suitability for a biventricular repair.

METHODS:

We reviewed the medical records for 106 patients with pulmonary atresia-intact ventricular septum (PA-IVS) treated between 1982 and 2001. Over this period, children were assigned to mild (> 2/3 normal size, 23.7% of patients), moderate (1/3 to 2/3, 41.2%), or severe (1/3, 35.1%) right ventricular hypoplasia, and this grouping, along with severity of coronary anomalies (45% right ventricle to coronary fistulae, 16% with right ventricle dependent coronary circulation [RVDCC]), triaged children to eventual single ventricle (severe) or two-ventricle (mild or moderate) repair.

RESULTS:

Actuarial 10-year survival was 86.3% with mortality predicted by severe hypoplasia (odds ratio [OR] 12.9, p < 0.001), RVDCC (OR 15.0, p < 0.001), and non-Caucasian race (OR 10.7, p < 0.001). Multivariate analysis with a Cox proportional hazards model confirmed only RVDCC (risk ratio [RR] 10.9, p = 0.0009} and non-Caucasian race (RR 6.9, p = 0.007) as significant. Although not an independent risk factor for survival, the degree of hypoplasia was the most important determinant for definitive repair. Severe hypoplasia virtually precluded two-ventricle repair (OR 33.1, p < 0.001 by chi2 analysis) and was the strongest risk factor for a one-ventricle system (OR 78.7, p < 0.001). Actuarial survival after either repair was 91%, and no biventricular repair later converted to a Fontan system.

CONCLUSIONS:

Surgical management of patients based on this three tier grade for right ventricular hypoplasia results in excellent survival and correctly predicts patients destined for eventual Fontan and biventricular repair.

• Authors: Jonah Odim, Hillel Laks, Mark D Plunkett, Thomas C Tung
• Journal: The Annals of thoracic surgery (Ann Thorac Surg) Vol. 81 Issue 2 Pg. 678-84 (Feb 2006) ISSN: 1552-6259 [Electronic] Netherlands
• PMID: 16427873 (Publication Type: Journal Article, Validation Study)

Topics

• Cardiac Surgical Procedures (methods)
• Female
• Heart Defects, Congenital (classification, surgery)
• Heart Ventricles (abnormalities, surgery)
• Humans
• Infant
• Infant, Newborn
• Male
• Prognosis
• Pulmonary Atresia (complications, surgery)
• Retrospective Studies
• Severity of Illness Index
• Sex Factors
• Survival Analysis