One and half ventricle repair: rationale, indications, and results

Abstract

Surgical strategies in patients with functionally or anatomically borderline right ventricles include a high-risk biventricular repair, a Fontan procedure, or a one and half ventricle repair (also referred to as the partial biventricular repair). One and half ventricle repair (1.5VR) circumvents the high early mortality of a biventricular repair and also the late morbidity of the Fontan. The two most common indications for a 1.5VR are a small pulmonary ventricle and a dilated poorly functioning pulmonary ventricle. Extension of 1.5VR to patients undergoing anatomical repair for congenitally corrected transposition of great arteries, straddling tricuspid valves, and severe Ebstein's anomaly has facilitated biventricular repair with decreased mortality. We reviewed the relevant literature on this subject in detail and describe its rationale, indications and its early and late results.

Keywords: Bidirectional Glenn; Complex biventricular repair; One and a half ventricle repair.

Fig. 1

Volume-rendered image of pulmonary circulation (Reproduced with permission from Uribe et al. [5])

Fig. 2

Particle traces emitted from the SVC show that blood flow is mainly directed to the RPA proximal to the d-RPA and the main PA (Reproduced with permission from Uribe et al. [5])

Fig. 3

In this heart with straddling and overriding of the tricuspid valve, the rudimentary right ventricle is markedly hypoplastic. It is unlikely to be suitable for supporting the entirety of the pulmonary circulation. (VSD—ventricular septal defect). (Reproduced with permission from Anderson and Ho [2])

Fig. 4

Initial CMR images at end-diastole phase: four-chamber view (a), two-chamber view of the right ventricle (b), two-chamber view of the left ventricle (c), and short-axis view of basal (d), mid (e), and apical segments (f). Right ventricular wall was overly thin and smooth due to absence of trabeculations, suggestive of right ventricular hypoplasia. The interventricular septum was deviated to the left ventricular side, indicating increased right ventricular end-diastolic pressure. Notably, the left ventricle showed prominent trabeculations. The increased noncompacted-to-compacted myocardium ratio suggested left ventricular noncompaction. Prominent epicardial fat deposits (asterisks) were also noted (Reproduced with permission from Kim et al. [16])

Fig. 5

Follow-up CMR of the patient described in Fig. 4. Four-chamber image (a) at end-diastole phase showed a severely distended right ventricle which deviated to the left ventricular side. Two-chamber of the right ventricle (b) and short-axis images (c) at end-systole phase showed poor coaptation of the tricuspid valve (black arrows) due to annular dilatation. Ten-minute delayed enhancement images (d–f) after gadolinium administration well demonstrated diffuse subendocardial or transmural enhancement (arrowheads) of the right ventricle. Notably, delayed enhancement was not observed in the basal lateral wall (open arrows) where the wall motion was preserved. Prominent epicardial fat deposits (asterisks) were still noted and appeared to have increased. (Reproduced with permission from Kim et al. [16])

Fig. 6

Surgical technique in patients with hypoplastic right ventricle. (Reproduced with permission from Kreutzer et al. [18])

Fig. 7

a Diagram of heart anatomy and function in a patient who had an atriopulmonary Fontan operation for presumed dextrocardia and tricuspid atresia. She was later found to have dextrocardia, crisscross heart, tricuspid stenosis, ventricular septal defect, severe pulmonary artery stenosis, normally related great arteries, baffle stenosis, and cyanosis with atrial re-entry tachycardia. b Diagram of repair after conversion from Fontan operation to in-parallel bidirectional Glenn shunt (11⁄2 ventricular repair) and modified right-sided Maze operation .(Reproduced with permission from Mavroudis et al. [22])

Fig. 8

Diagrammatic representation of a patient with looped transposition of the great arteries, ventricular septal defect, and pulmonary stenosis who had ventricular septal defect closure, pulmonary valvulotomy, and in-parallel bidirectional Glenn shunt to volume unload the high pressure in the left ventricle (pulmonary ventricle). (Reproduced with permission from Mavroudis et al. [22])

Fig. 9

The probability of keeping an interatrial shunt of the fenestrated atrial septal defect (ASD) cases.(Reproduced with permission from Kim S et al. [29])

Fig. 10

a Kaplan-Meier curve for cumulative survival of the 105 late survivors described by Kim et al. b Kaplan-Meier curve for cumulative survival according to the operative era.(Reproduced with permission from Kim S et al. [29])