A 3-year-old girl was transferred to our hospital to determine the indication for heart transplantation. The patient was born at 41 weeks of gestation weighing 2.8 kg. Due to respiratory failure, she was admitted to the referring hospital where she was diagnosed with truncus arteriosus type A2. Bilateral PA banding was performed on the 4th day after birth, and the patient was discharged on postoperative day 17. Two months after her birth, palliative RVOTR with the LeCompte maneuver was performed using an 8-mm hand-made polytetrafluoroethylene (PTFE) tri-leaflet valved conduit. The postoperative course was uneventful, and the patient was discharged on postoperative day 13. Six months after her birth, the patient was urgently admitted to the original referring hospital due to progressive deterioration in her general condition. Echocardiogram showed severe truncal valve regurgitation and reduced LV function. Truncal valve replacement with the Konno procedure using the 16 mm ATS Advanced Performance valve (ATS Medical Inc., MN, USA) and atrial septal defect enlargement was emergently performed. Because of the hypoplastic pulmonary arteries and LV dysfunction, ventricular septal defect closure was not performed at this stage. The postoperative course was complicated with atrial arrhythmia and LV dysfunction. The patient was treated with amiodarone and an angiotensin-converting enzyme inhibitor, and she was discharged on postoperative day 83. At 2 years of age, a systemic-to-PA shunt was constructed because of progressive hypoxia. At 3 years of age, the patient developed acute progression of heart failure triggered by influenza type A infection. Considering the possibility of low coronary flow, the systemic-to-PA shunt was embolized with coils. For severe bilateral PA stenosis, balloon dilatations were performed for both PAs. The sizes of the right and left PAs after the procedure were 4.3 mm and 4.5 mm, respectively, and the pressure gradient between peripheral PA and PA trunk was 47 mmHg (Fig. 1). The pressures of right PA, left PA, main PA, and right ventricle were 11/2(7) mmHg, 26/6(17), 47/15(29) mmHg, and 84/edp 7 mmHg, respectively. Despite maximum treatment, the patient’s cardiac function did not recover, and the patient’s parents opted for heart transplantation.

Pulmonary angiogram before transfer to our center

The patient was intubated and transferred to our center by medical jet. On admission, echocardiography showed biventricular dysfunction with an LV ejection fraction of 8% and an RV fractional area change of 28% with inotropic support. Brain natriuretic peptide was markedly elevated to 3131.7 pg/mL. On the 2nd day after admission, signs of multiorgan failure appeared, and emergent biventricular-assist device (BiVAD) implantation was performed. The body size at this surgery was as follows: height of 91 cm, body weight of 10.8 kg, and body surface area of 0.51m2. During surgery, truncal valve closure, ventricular septal defect closure, atrial septal defect closure, and re-RVOTR using the 14 mm hand-made PTFE tri-leaflet conduit were concomitantly performed. Regarding truncal valve closure, we removed the mechanical valve (retaining the cuff of it), and then performed a patch closure onto the cuff using a 0.4 mm PTFE patch. Ventricular septal defect closure with 0.4 mm PTFE patch and direct atrial septal defect closure were performed. The central PA was reconstructed behind the ascending aorta using an 8-mm PTFE conduit. Given the impact of residual pulmonary stenosis, a temporary RV-assist device (RVAD) was required. In terms of the VADs, centrifugal pumps were used for both ventricles with an artificial lung in the LVAD circuit. For LVAD cannulae, we chose a 6-mm apex cannula (C18A-020) as inflow cannula and a 6-mm arterial cannula (C19V-020) as outflow cannula. Instead, for RVAD, we inserted 16-Fr inflow cannula (LARGE FLOW® VENOUS RETURN CANNULA) and 10-Fr outflow cannula (Bio-Medicus® NextGen arterial cannula) into IVC via RA and distal pulmonary artery trunk (namely, distal side of the hand-made tri-leaflet valve of the conduit), respectively, both with a purse-string suture. Figure 2 illustrates the surgical procedure. Although postoperative cardiac catheterization showed severe PA stenosis with a pressure gradient of 85 mmHg, biventricular support was effective at stabilizing the patient’s circulation. On postoperative day 7, the RVAD was successfully removed and right PA plasty was performed. On postoperative day 14, the artificial lung was successfully removed. Cardiac catheterization was performed on postoperative day 19, which showed pressure gradients for right and left pulmonary stenosis of 61 mmHg and 83 mmHg, respectively, after PA balloon dilatation. On postoperative day 30, catheter PA balloon dilatation was performed again for pulmonary stenosis. The pressure gradient for right and left pulmonary stenosis decreased to 40 mmHg and 32 mmHg, respectively; namely, the pressures of right PA, left PA, main PA, and right ventricle were 28/15(20) mmHg, 41/16(27), 72/14(37) mmHg, and 78/edp 13 mmHg, respectively. After obtaining in-house approval for heart transplant registration, the LVAD was converted to the BHE (pump size of 15 ml) 35 days after BiVAD implantation. The patient was extubated 3 days after the conversion and discharged from the intensive care unit 5 days after extubation. Cardiac catheterization performed 2 months after intensive care unit discharge showed an improvement in pulmonary stenosis with a pressure gradient of 20 mmHg. The sizes of the right and left PAs were 6.4 mm and 8.6 mm, respectively, and the pressure gradient between peripheral PA and PA trunk was 25 mmHg (Fig. 3). The patient was registered in the Japanese organ transplant network and is waiting for a donor organ in a stable condition.

Illustration of the surgery. A Before the surgery, B after the surgery

Pulmonary angiogram after Berlin Heart EXCOR implantation