Role of Modified Right-Ventricular Overhaul Procedure in Pulmonary Atresia with Intact Ventricular Septum: Promising Effect on Growth of Right Ventricle Based on Magnetic Resonance Imaging Study

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Abstract Submission 

Jae Hong Lee (1), Woong-Han Kim (2), Hye Won Kwon (2), Sungkyu Cho (3), Jae Gun Kwak (4)

(1) Pusan National University Yangsan Hospital, Yangsan-si, (2) Seoul National University Children's Hospital, Seoul, (3) Seoul National University Children's Hospital, SEOUL, (4) Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul,

Jae Hong Lee    -  Contact Me
Pusan National University Yangsan Hospital

Woong-Han Kim    -  Contact Me
Seoul National University Children's Hospital

Hye Won Kwon    -  Contact Me
Seoul National University Children's Hospital

Sungkyu Cho    -  Contact Me
Seoul National University Children's Hospital

Jae Gun Kwak    -  Contact Me
Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital

Jae Hong Lee    -  Contact Me
Seoul National University Children's Hospital

Objective: Pulmonary atresia with intact ventricular septum(PA-IVS) has a broad spectrum of treatment strategies ranging from single ventricle palliation to biventricular repair. We aimed to evaluate the clinical outcomes of patients who underwent our modified RV overhaul(mRVoh) procedure.
Methods: We conducted a retrospective review of 25 cases in 23 patients with PA-IVS without RV-dependent coronary circulation who underwent the mRVoh procedure between 2008 and 2023. The operative strategy of the mRVoh procedure for PA-IVS included wide resection of hypertrophied infundibular and trabecular muscle with peeling off fibrotic endocardial tissue in the RV cavity, surgical pulmonary valvotomy and tricuspid valve(TV) repair. In the neonatal and young infant patients, we performed B-T shunt or temporary PDA banding simultaneously and considered PDA stenting depending on the antegrade pulmonary flow after the mRVoh procedure. The z-scores of TV and PV annulus, as well as valve function, were assessed by echocardiography. RV function and volume index were measured by magnetic resonance imaging(MRI).
Results: The median age and body weight at the mRVoh procedure were 7.0months and 7.6kg, respectively. Seven were neonates and three were young infants(< 3months). Twenty underwent intervention or surgery for PA-IVS before the mRVoh procedure. Four finally underwent 1.5-V repair; of these, three underwent 1.5-V repair before the mRVoh procedure. There were no early- and late mortalities. Three patients required reoperation(re-do mRVoh procedure in two and TV repair in one). During the median follow-up of 2.25years, the median TV annular z-score tended to increase from -2.53 to -1.01, and tricuspid regurgitation grade improved significantly(1.5 vs.1.0,p=0.041) before and after the mRVoh procedure. In the subgroup study of ten who underwent two MRIs at median intervals of 2.8years after the mRVoh procedure, MRI studies showed that the median RVEDVI (49.2[IQR,33.3-62.8]vs.87.1[IQR,69.4-111.2],p=0.005), RVESVI (19.6[IQR,14.6-35.0]vs.42.2[IQR,33.0-50.7], p=0.005), and RV cardiac output index(2.8[IQR,2.1-3.4]vs.4.1[IQR,3.13-4.65],p=0.007) had significantly increased, respectively.
Conclusions: We observed the effective growth of RV, including TV annulus, with acceptable PV function after mRVoh which ultimately could lead to 2-V repair. Our results showed that mRVoh procedure might be considered as feasible option in terms of RV growth in selected patients with PA-IV

Reconstruction of Complex Congenital Heart Disease