Outcome of congenital heart disease with left-to-right shunts and pulmonary arterial hypertension undergoing operability assessment by acute vasodilator testing: The AIIMS operability score.

BACKGROUND

Pulmonary artery hypertension associated with left-to-right intracardiac shunts adversely affects outcomes. Studies are limited to guide the decision to operate or not in patients presenting late with a left-to-right shunt and PAH. This study aimed to assess clinical and investigative parameters, including acute vasodilator testing (AVT) using oxygen, to predict operability decisions and outcomes.

MATERIALS AND

METHODS

We conducted a retrospective analysis of patients who underwent cardiac catheterization for operability assessment over 5 years, excluding those with left-heart obstructive disease or residual postoperative lesions. A total of 157 patients (mean age: 12.8 ± 12.6 years; 83 males) were included, with the majority having post-tricuspid shunt lesions (132/157, 84%). Multiple logistic regression identified predictors of inoperability on AVT, including age >2 years, absence of cardiomegaly, bidirectional or predominantly right-to-left shunting across the defect, and absence of left ventricular volume overload on echocardiography. These variables formed a scoring system with 80% specificity and 60% sensitivity for predicting inoperability.

RESULTS

Of the 157 patients, 60 underwent surgical correction, with six experiencing right heart failure or death post-operatively, and three facing adverse outcomes during further follow-up. Univariate analysis indicated that age >2 years, hemoglobin levels, single or narrowly split second heart sound, cyanosis, and a postoxygen Qp: Qs ratio <0.9 were significantly associated with unfavorable outcomes among patients who underwent surgery. In the multivariate analysis, none of the parameters predicted an unfavorable surgical outcome.

CONCLUSIONS

This study highlights the importance of simple clinical and non-invasive parameters in assessing operability among patients with left-to-right intracardiac shunts and severe PAH. However, none of the parameters were independently predictive of operative outcome. A simple AIIMS operability score based on clinical and noninvasive parameters can predict inoperability with acceptable sensitivity and specificity. However, the score should be validated in larger prospective studies.