Multidisciplinary Approach for Establishing a 1.5 V or Biventricular Circulation in Patients with Complex Congenital Heart Disease

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All articles of this category (opens in new window) Background: Patients with a failing Fontan circulation are at increased risk for mortality and morbidity. This has led to the pursuit of approaches aiming at establishing a 1.5 ventricle (1.5 V) or biventricular (BiV) circulation. Here, we describe our institutional experience with staged 1.5 V or BiV repair for complex congenital heart disease (CHD) in five patients. Methods: Patients 1 and 2 were in Glenn circulation at the time of clinical decision-making. The underlying cardiac diagnosis was a double outlet right ventricle (DORV) with a common atrioventricular septal defect (cAVSD) and hypoplastic left ventricle (LV) and superior-inferior ventricles with a hypoplastic LV in patients 1 and 2, respectively. Patient 3 was transferred to our institution with a failing Fontan circulation. Cardiac diagnosis was congenitally corrected transposition of great arteries (ccTGA) with hypoplastic LV. The other two patients with hypoplastic left heart complex with hypoplastic LV, mitral and aortic valve (MV; AV), and ccTGA with hypoplastic RV underwent a hybrid procedure with pulmonary flow restrictors and ductal stenting to allow growth of the hypoplastic ventricles. All patients underwent CT, 3D reconstruction of the CT dataset, and cardiac catheterization. In addition, ⅘ patients received an MRI for the determination of ventricular volumes. Patient 2 underwent an EP study and intraoperative mapping (IOM) for identification of the exact course of the distal conduction system. Results: Median volumes of the hypoplastic ventricle were 40.4 mL/m 2 . A 1.5 V or BiV repair was successful in all patients: patients 1 and 2 underwent 1.5 V repair with hemi-mustard and establishment of a LV-PA conduit. Patient 3 underwent Fontan-Take-Down and establishment of a physiological correction with an LV-PA conduit. BiV repair with reconstruction of the aortic arch and MV and double switch operation with hemi-mustard and establishment of Glenn circulation was performed in patients 4 and 5, respectively. Postoperatively, patient 1 developed complete heart block (CHB) requiring pacemaker implantation. Patient 3 had a stable junctional escape rhythm with no need for pacemaker implantation. All other patients had sinus rhythm. No additional major complications were noted. All patients were discharged in a clinically stable condition. Conclusion: A concise multidisciplinary approach with interventionalists, surgeons, clinicians, radiologists, and electrophysiologists allows better finding of individualized therapy in complex CHD, especially 1.5 V and BiV repair in patients on a palliative care pathway. Furthermore, IOM demonstrated promising results in avoiding surgery-related CHB and may improve outcomes. Close follow-up is required to determine the outcome of these patients. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

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