Right ventricle to pulmonary artery conduit: a comparison of long-term graft-related events between bovine jugular vein conduit, aortic homograft, and porcine-valved conduits.

Background: The optimal conduit for right ventricle to pulmonary artery (RV-PA) reconstruction does not exist. Reinterventions are common due to conduit stenosis and endocarditis. Tailoring conduit choice according to patients' characteristics could improve the outcomes. The study aimed to compare graft-related events (infective endocarditis, transcatheter pulmonary valve replacement (PVR), transcatheter conduit dilatation, surgical conduit replacement, and transcatheter pulmonary branch intervention for RV-PA reconstruction using bovine jugular vein, aortic homograft, and porcine-valved conduits. Methods: In a retrospective cohort study, 155 patients with 193 procedures that were done in King Faisal Specialist Hospital and Research Center-Jeddah (KFSHRC JED) for implanting RV-PA conduits from 1999 to 2021 were included. The patients were grouped according to the type of conduit into 3 groups; Group 1 (n=153) received bovine jugular vein (BJVs) grafts, Group 2 (n=29) received aortic homografts, Group 3 (n=11) received porcine-valved conduits. Factors associated with graft-related events were evaluated using stepwise Cox regression analysis. Results: Patients with BJVs were significantly younger than those with porcine-valved conduits 3 (P=0.009). The weight of BJVs patients was significantly lower than homografts (P=0.002) and porcine-valved conduits patients 3 (P<0.001). The conduit size was as expected significantly lower in BJVs patients than patients who received porcine-valved conduits (P<0.001) and patients who received aortic homografts (P<0.001). There was no difference between Group 2 and 3 (P=0.084). Operative mortality occurred in 13 patients: 12 (7.84%) with BJV conduit and 1 (9.09%) with porcine valved conduit (P=0.351). Male gender [odds ratio (OR): 10.04; 95% confidence interval (CI): 1.28-78.86; P=0.028] and smaller conduit size (OR: 0.78; 95% CI: 0.61-0.99; P=0.048) were associated with increased operative mortality. Freedom from graft-related events at 5 and 10 years was 67% and 52% in BJVs patients, 74% and 36% in patients who received aortic homografts, and 53% in patients who received porcine-valved conduits. Factors associated with increased graft-related events were male gender (HR: 1.58; 95% CI: 1.004-2.50, P=0.048) and younger age (HR: 0.995; 95% CI: 0.991-0.999, P=0.041). Conclusions: RV-PA reconstruction was associated with low mortality, unrelated to the conduit type. Reinterventions for graft-related events were common. The durability and graft-related events might be comparable among BJV grafts, aortic homografts, and porcine-valved conduits. Factors associated with increased graft-related events in this study were male gender and younger age.

Patent ductus arteriosus stenting versus modified Blalock-Taussig shunt for palliation of duct-dependent pulmonary blood flow lesions.

BACKGROUND: Patent ductus arteriosus (PDA) stenting is an alternative to modified Blalock-Taussig shunt (MBTS) as first-stage palliation of duct-dependent lesions. The superiority of one approach over the other is still controversial. Our objective was to compare PDA  stent versusMBTS for palliation in regard to safety, efficacy, and efficiency. METHODS: From 2010 to 2021, 134 patients had first-stage palliation with either PDA stent (n = 83) or MBTS (n = 51). Twenty-seven patients failed the primary treatment and were converted to the other group. The study endpoints were hospital outcomes, interstage reintervention, and concomitant procedures at the second-stage palliation. RESULTS: Patients with PDA stent were significantly younger. The prevalence of antegrade pulmonary blood flow (PBF) was higher in patients who had MBTS and graft thrombosis was higher in the PDA stent. Hospital stay was significantly longer in patients who had MBTS. Predictors of prolonged mechanical ventilation were low-weight, MBTS, and conversion. Intensive care unit stay significantly increased with conversion, low-weight, and antegrade PBF. The interstage intervention was required more frequently in PDA-stent group. Predictors of reintervention were conversion and pulmonary atresia with the intact interventricular septum. Pulmonary artery plasty was required more frequently during the second-stage palliation in PDA-stent group. CONCLUSION: PDA stent is an alternative to MBTS for first-stage palliation. It is associated with shorter hospital stays and avoidance of surgery at the expense of a high rate of stent thrombosis and interstage reintervention. Conversion increased the risk of the procedure. More studies are needed to determine factors that affect PDA-stent outcomes and patient selection criteria.

Safety and efficacy of transcatheter closure of atrial septal defect type II under transthoracic echocardiographic guidance: A case control study.

BACKGROUND: Transcatheter closure of secundum atrial septal defect is routinely performed under general anesthesia and transesophageal echocardiography guidance. If patients have good echo windows, the procedure could be performed under transthoracic echo guidance. AIM OF STUDY: To evaluate safety and efficacy of the intervention using fluoroscopy and echo guidance. METHODS: In a case control study design, 180 patients underwent atrial septal defect closure between January 2010 and December 2016. In 32 patients, the intervention was performed under fluoroscopy and transthoracic echo guidance. Our study group consisted of 22 out of 32 patients (<13 years old). For the other 10 patients, we could not find a matching pair. The data of the study group were compared with an age, weight, and height matched group (controls), who underwent the procedure under transesophageal echocardiography guidance. RESULTS: The diameter of the atrial septal defect, septal length, and most of the rims were comparable. The superior rim and inferior rims were longer in the study group. The devices chosen for the cases were larger than the control group. Procedure time and fluoroscopy times were shorter in the study group. Success rate was comparable. On follow-up, both groups had almost no or minimal incidence of residual shunt. CONCLUSION: We conclude that transcatheter closure of atrial septal defect under fluoroscopy and transthoracic echo guidance is safe and successful in selected patients who have single central atrial septal defect with adequate septal lengths and adequate septal rims, with high incidence of complete occlusion rate.

Recanalization of arterial duct is feasible, effective and its potential risks are treatable.

BACKGROUND: Recanalization of arterial duct (AD) is rarely needed. OBJECTIVE: The aim of this study is to report our experience regarding the feasibility and effectiveness of arterial duct recanalization in three infants and review the relevant literature. METHODS AND RESULTS: We report on three patients with decreased pulmonary blood flow after initial palliation. The first patient had pulmonary atresia (PA) and an intact ventricular septum. He needed recanalization of the AD after pulmonary valve perforation and dilatation. The second patient had PA and ventricular septal defect (VSD). His AD originated from the left subclavian artery. He required AD recanalization after palliation with a central shunt and clipping of the duct. During intervention he developed a thrombus in the stent, which was treated successfully using thrombolytic treatment. The third patient had PA and VSD. The arterial duct originated from the left subclavian artery and was recanalized after spontaneous closure despite prostaglandin infusion. During the procedure the patient had severe desaturation and bradycardia requiring resuscitation fortwo minutes. All infants had successful arterial duct recanalization and stenting. Additionally, they were clinically stable during follow-up. CONCLUSION: Arterial duct recanalization and stenting is a feasible and effective procedure in selected cases, and its risks are treatable. Long-term studies are required.

Alpha blocker and angiotensin-converting enzyme inhibitor in the management of severe pulmonary valve stenosis: from bench to bedside.

INTRODUCTION: Neonates with severe pulmonary valve stenosis tend to remain oxygen dependent, despite resolution of the transpulmonary gradient. Alpha 2 blockers ­ phentolamine ­ and angiotensin-converting enzyme inhibitors ­ captopril ­ were reported to improve oxygen saturation. OBJECTIVE: To describe the role of phentolamine and captopril in the treatment of these patients. METHODS: In a retrospective cohort study, 28 neonates with severe pulmonary valve stenosis underwent balloon valvuloplasty. Among them, 20 remained oxygen or prostaglandin dependent after intervention, and were treated with phentolamine or captopril. Oxygen saturation was monitored before and after intervention and following treatment with these medications. Mean duration of hospitalisation was recorded. RESULTS: Mean age and weight were 25.2 days and 3.1 kg, respectively. Before balloon dilation, 18/20 (90%) neonates were on prostaglandin, whereas after the procedure only 6/18 patients required it. All 20 patients required oxygen after the procedure, and nine patients (45%) were started on phentolamine. Among them, one patient with severe infundibular stenosis did not respond favourably, and 11 patients (55%) were started on captopril. After starting phentolamine or captopril treatment, prostaglandin could be discontinued after a mean time of 15.86 hours. Within <2 days, there was an increase in mean oxygen saturation from 76.6 to 93.0%. CONCLUSION: Phentolamine and captopril seem to have therapeutic roles in neonates with severe pulmonary valve stenosis who remain oxygen dependent after balloon dilation. Both drugs led to vasodilation of the pulmonary and systemic vascularisation and facilitated inflow to the right ventricle. Right-to-left shunt across a patent foramen ovale or atrial septal defect decreased and saturation improved, leading to a significant reduction in the length of hospitalisation.

Post Melody valve implant in a conduit with regurgitant native outflow tract successful closure by Amplatzer muscular device.

We describe the technique of closure of native right ventricular outflow tract by Amplatzer muscular ventricular septal defect device because of severe regurgitation in a patient who had tetralogy of Fallot repair with conduit at 3 years of age followed by percutaneous Melody valve implant 6 years later.

Cardiac involvement in geleophysic dysplasia in three siblings of a Saudi family.

Geleophysic dysplasia is an extremely rare acromelic skeletal dysplasia resembling lysosomal storage disease. It is characterised by characteristic facial phenotype, short stature, micromelia, joint contracture, and early cardiac valvular involvement. It has been described worldwide in <40 patients. Herein, we describe the cardiac features in three Saudi sisters with proved autosomal recessive geleophysic dysplasia who showed different levels of severity of their cardiac involvement.

Cardiac rhabdomyoma mimicking haemodynamics of hypoplastic left heart syndrome.

Cardiac rhabdomyomas are rare and often regress spontaneously. However, the management of rhabdomyoma with severe inflow and outflow obstructions is a challenge. An infant with a massive left ventricular rhabdomyoma mimicking the haemodynamics of hypoplastic left heart syndrome is reported. Surgery could not be contemplated because the mitral valve leaflets and chordae were imbedded in the tumour mass. The arterial duct (AD) was kept open to perfuse the systemic circulation and palliations with pulmonary artery branch banding and AD stenting were planned. However; while waiting for spontaneous regression of the tumour, the child died of circulatory collapse when 4 weeks old.

Successful percutaneous closure of a secundum atrial septal defect through femoral approach in a patient with interrupted inferior vena cava.

Percutaneous closure of secundum atrial septal defect (ASD II) is considered the treatment of choice in the majority of cases. Interrupted inferior vena cava with azygos continuation can make delivery of the occluder difficult or not possible. Transjugular, transhepatic approach or surgery can be the alternative. We present the case of a 53-year-old woman with ASD II and interrupted inferior vena cava, and describe successful atrial septal defect closure under transesophageal echocardiography guidance through transfemoral approach using a modification of the standard technique.

Resternotomy for a retrosternal cardiac pseudoaneurysm in a 1.5-year- old child: a case report.

Cardiac pseudoaneurysm is a contained rupture of the myocardium that can occur after cardiac surgery, chest trauma, and endocarditis. The wall of the pseudoaneurysm consists of fibrous tissue and lacks the structural elements found in a normal cardiac wall, and it is contained by the pericardial adhesions or the epicardial wall. Early surgery is recommended even for asymptomatic patients due to the propensity for rupture and fatal outcome. We report our experience with the surgical approach of a child with a cardiac pseudoaneurysm who had undergone a biventricular repair of a double outlet right ventricle with non-committed ventricular septal defect in the form of intraventricular tunneling.