Long-Term Outcomes After an Individualized Strategy in Patients with Pulmonary Atresia and Intact Ventricular Septum.

This retrospective cohort study aimed to explore the long-term outcomes of an individualized strategy in patients with pulmonary atresia and intact ventricular septum (PAIVS). We analyzed survival and reintervention rates and identified risk factors for outcomes in patients with PAIVS treated based on individual right heart structures between 1979 and 2019. Ninety-five patients were included in this study. The z-scores of the pulmonary annulus, tricuspid annulus, and right ventricular end-diastolic volume were - 3.30 (- 15.15 to 1.83), - 0.70 (- 4.65 to 2.33), and - 1.51 (- 6.35 to 1.18), respectively. Right ventricular-dependent coronary circulation occurred in 15% of the patients. Among the 63 patients attempting biventricular strategy at first, 55 patients achieved biventricular circulation, 3 patients had one-and-a-half circulation, and 4 patients died perioperatively. Among the 33 patients attempting univentricular strategy at first, 10 patients died before the completion of Fontan operation, 17 patients (48%) accomplished Fontan operation, and 5 patients waited for Fontan operation. In one patient, conversion to biventricular circulation occurred. During the follow-up period of 720 person-years, the 20-year survival rate was significantly higher in patients with biventricular circulation than in those patients with univentricular circulation (93% vs. 67%, P < 0.001). Freedom from reintervention rates at 20 years was significantly lower in patients with biventricular circulation than in those patients with univentricular circulation (29% vs. 72%, P < 0.001). The pulmonary annulus z-score was an independent risk factor for reintervention in patients with biventricular circulation. Patients with biventricular circulation had an acceptable survival rate, but a high reintervention rate. Meanwhile, patients with univentricular circulation had high mortality before the completion of Fontan operation, although the reintervention rate was relatively low.

Fetal Cardiac Interventions-Are They Safe for the Mothers?

The aim of fetal cardiac interventions (FCI), as other prenatal therapeutic procedures, is to bring benefit to the fetus. However, the safety of the mother is of utmost importance. The objective of our study was to evaluate the impact of FCI on maternal condition, course of pregnancy, and delivery. 113 mothers underwent intrauterine treatment of their fetuses with critical heart defects. 128 percutaneous ultrasound-guided FCI were performed and analyzed. The patients were divided into four groups according to the type of FCI: balloon aortic valvuloplasty (fBAV), balloon pulmonary valvuloplasty (fBPV), interatrial stent placement (IAS), and balloon atrioseptoplasty (BAS). Various factors: maternal parameters, perioperative data, and pregnancy complications, were analyzed. There was only one major complication-procedure-related placental abruption (without need for blood products transfusion). There were no cases of: procedure-related preterm prelabor rupture of membranes (pPROM), chorioamnionitis, wound infection, and anesthesia associated complications. Tocolysis was only necessary only in two cases, and it was effective in both. None of the patients required intensive care unit admission. The procedure was effective in treating polyhydramnios associated with fetal heart failure in six out of nine cases. Deliveries occurred at term in 89%, 54% were vaginal. The results showed that FCI had a negligible impact on a further course of pregnancy and delivery.

Fetal Cardiac Interventions-Polish Experience from "Zero" to the Third World Largest Program.

This article presents the technical aspects of the Polish fetal cardiac interventions (FCI) program, including preparation of the team and modifications in the technique of the procedure that aim to increase its safety for the mother and the fetus. Over 9 years, 128 FCI in 113 fetuses have been performed: 94 balloon aortic valvuloplasties (fBAV), 14 balloon atrioseptoplasties (fBAS) with stent (BAS+), 5 balloon atrioseptoplasties without stent placement (BAS-), and 15 fetal pulmonary valvuloplasties (fBPS). The technical success rate ranged from 80% (BAS-) to 89% (fBAV), while the procedure-related death rate (defined as death within 72 hours following the procedure) ranged from 7% (fBAV and fBPV) to 20% (BAS). There were 98 live births after all FCI (3 pregnancies continue). Median gestational age at delivery was 39 weeks in our center and 38 weeks in other centers.

How to deal with atrial septal defect closure from right internal jugular vein: Role of venous-arterial circuit for sizing and over-the-wire device implantation.

Secundum atrial septum defect (ASD) is the most common congenital heart disease. It is usually treated by a transcatheter approach using a femoral venous access. In case of bilateral femoral vein occlusion, the internal jugular venous approach for ASD closure is an option, in particular in cases where ASD balloon occlusion test and sizing is needed. Here, we report on a new technique for ASD closure using a venous-arterial circuit from the right internal jugular vein to the femoral artery. Two patients (females, 4 and 10 years of age) had occlusion of both femoral veins because of a previous history of pulmonary atresia and intact ventricular septum, for which they underwent percutaneous radiofrequency perforation and balloon angioplasty. These subjects needed balloon occlusion test of a residual ASD to size the hole and to check for hemodynamic suitability to ASD closure. After performing a venous-arterial circuit, a 24 mm St Jude ASD sizing balloon catheter was advanced over the circuit and the defect closed for 15 min to check hemodynamics and size the defect. ASD was closed is hemodinamically suitable. This technique was safe and reliable. © 2016 Wiley Periodicals, Inc.

A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.

OBJECTIVES: To achieve the growth of right-sided heart structures, our choice of the first palliation for patients with pulmonary atresia and intact ventricular septum (PA-IVS) includes a modified Blalock-Taussig shunt (BTS) with pulmonary valvotomy. We sought to analyse the impact of the first palliation on the growth of right-sided heart structures and factors associated with a choice of definitive surgical procedure. METHODS: Fifty patients with PA-IVS who underwent a staged surgical approach from 1991 to 2012 were retrospectively reviewed. RESULTS: Right ventricular (RV)-coronary artery fistulas were seen in 42% of patients at the time of birth. All 50 patients had a modified BTS with pulmonary valvotomy. Six patients died after the first palliation or inter-stage. Thirty patients achieved a biventricular repair (BVR group), 6 patients had a 1 + 1/2 ventricular repair (1 + 1/2V group) and 5 patients had a Fontan completion (Fontan group). After modified BTS with pulmonary valvotomy, tricuspid valve z-score did not increase in any of the group (BVR: pre -2.79 vs post -2.24, 1 + 1/2V: pre -5.25 vs post -6.69, Fontan: pre -6.82 vs post -7.94). Normalized RV end-diastolic volume increased only in BVR group after modified BTS with pulmonary valvotomy (BVR: pre 32% vs post 64%, 1 + 1/2V: pre 43% vs post 42%, Fontan: pre 29% vs post 32%). Major RV-coronary artery fistula was a strong factor with proceeding single-ventricle palliation [BVR: 4/30 (13%) patients, 1 + 1/2V: 1/6 (17%) and Fontan: 4/5 (80%)]. CONCLUSIONS: Tricuspid valve growth was not obtained by modified BTS with pulmonary valvotomy; therefore, tricuspid valve size at birth appeared to be a predictor for achieving BVR. Proportionate RV growth was seen only in patients who achieved BVR. However, RV growth was not seen in patients having 1 + 1/2 ventricular repair. Major RV-coronary artery fistula was a strong predictor for proceeding single-ventricle palliation.