Reversibility and outcome of fenestrated surgical closure in adult patients with congenital left-to-right shunts and pulmonary hypertension: a result of cohard-ph registry

Abstract

Abstract Background Uncorrected congenital heart disease in the adults often come with a complication of pulmonary hypertension (PH) , resulting in high mortality and low quality of life due to right heart failure and arrythmias. Shunt closure in those patients has been shown to reduce such complications. However, in patients who develop PH, total shunt closure posseses risk that increase mortality. ESC guidelines recommend avoidance of ASD closure and careful evaluation of VSD and PDA closure in those with PVRI>5 WU. Nevertheless, some Asian countries reported good outcome in LtoR shunt closure in patients with PVRI>5. Congenital Heart Disease in Adults and Pulmonary Hypertension (COHARD-PH) Registry is a single tertiary center registry in Indonesia. The data showed that among congenital LtoR shunts, PAH occurred in 66.9% patients and Eisenmenger Syndrome in 18.7%. In this study we aim to evaluate the outcome and the PH reversibility of adult patients underwent fenestrated closure in this registry. Methods Patients with intracardiac shunts who underwent surgical closure with atrial septal fenestration on January 2019-July 2022 were recruited and the follow-up data was obtained within 6 months after surgery. Results Thirty-eight patients underwent surgical fenestrated closure were identified. Age was ranging between 19-70 (mean 39) years-old and 81.6% were female. Thirty patients (30%) were ASD, 7 (18.4%) were VSD, and 1 patient (2.6%) was incomplete AVSD. All patients underwent 4-5 mm atrial septal fenestration despite the repair of their anatomical lesion. Hemodynamic measurement before surgery showed that the average Qp/Qs was 3.2; mPAP 51±17 mmHg and the mean pulmonary vascular resistance index (PVRI) was 7.2±4.4 WU before surgery. We performed acute vasoreactivity test (AVT) to 21 patients and showed positive result, while the rest of the patients did not have AVT due to PVRI of 4 WU or less. Short and mid-term follow up reveal a mean SICU length of stay was 2.5 days and mean total hospital stay was 9 days. Three patients died during their hospital stay after discharged from the SICU due to post operative sepsis. On 6 month follow up, the remaining 35 subjects have no remaining symptoms. The reversibility of PH was assesed by echocardiography and showed that mean TVG was reduced from 78 to 36 (by 54%). None of these patients showed signs of heart failure or pulmonary hypertension on follow up. Conclusion Atrial septal fenestration in patients with adult congenital left to right shunt undergoing surgical closure might be considered in selected patients with pulmonary hypertension including those with positive acute vasoreactivity test. Further study with larger subjects is warranted to evaluate the current guidelines.