Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome.

OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart disease with severe airway compression due to dilatation of the pulmonary arteries (PAs). We investigated risk factors for death and prolonged mechanical ventilation (>7 days) and a threshold PA size for these outcomes. METHODS: This retrospective 2-centre cohort study included 68 patients with complete repair between January 1996 and December 2015. RESULTS: Median age at repair was 3.9 months (1.3-8.7 months), and median weight was 5 kg (4-7 kg). The mortality rate before hospital discharge was 12%, and the mortality rate at last follow-up was 19%. In multivariable analysis, risk factors for death were higher Nakata index [hazard ratio (HR) 1.001, 95% confidence interval (CI) 1.001-1.002; P < 0.001] and lower SpO2 (HR 1.06, 95% CI 1.02-1.09; P = 0.002). The accuracy of the Nakata index to predict death was excellent (area under the curve at 6 months: 0.92; P = 0.010). A Nakata index above 1500 mm2/m2 predicted mortality at 6 months with a sensitivity of 98% and a specificity of 82%. Twenty-five patients (37%) had prolonged mechanical ventilation. The only multivariable risk factor for prolonged ventilation was lower weight at repair (odds ratio 2.9, 95% CI 1.3-6.7; P = 0.008). Neither PA plasty nor the LeCompte manoeuvre had a protective effect on mortality or prolonged ventilation. A Nakata index above 1500 mm2/m2 remained a risk factor for mortality (P = 0.022) in patients who had a PA plasty or the LeCompte manoeuvre. CONCLUSIONS: In patients with absent pulmonary valve syndrome, the Nakata index predicts mortality with a cut-off of 1500 mm2/m2. Lower weight at repair is the only multivariable risk factor for prolonged ventilation. Neither PA plasty nor the LeCompte manoeuvre had a protective effect on these outcomes.

Percutaneous pulmonary Melody® valve implantation in small conduits.

BACKGROUND: The Melody® pulmonary valve has received approval for the treatment of dysfunctional right ventricular (RV) outflow tract conduits ≥16mm. AIMS: To investigate technical and clinical outcomes in patients who underwent percutaneous pulmonary valve implantation (PPVI) in conduits ≤16mm. METHODS: Eleven patients were enrolled retrospectively between 2000 and 2015 in a cardiac referral centre. RESULTS: The PPVI indications were obstruction (n=5); regurgitation (n=1); and mixed lesions (n=5). Pre-stenting was performed during the procedure in seven cases. Three of the remaining four patients had previous stent implantation. The mean maximal dilatation balloon diameter/surgical initial diameter ratio was 1.3. Procedural haemodynamics showed a decrease in peak RV to pulmonary artery gradient (mmHg) from 50 (14-86) to 11 (5-23) (P<0.001), in RV systolic pressure (mmHg) from 69 (35-110) to 40 (25-50) (P=0.006) and in RV/aortic systolic pressure ratio from 0.8 (0.4-1.0) to 0.4 (0.3-0.6) (P=0.004). Acute device-related complications occurred in four patients (36.4%): two confined conduit tears managed with placement of a covered stent; and two vascular access complications. Mean follow-up after PPVI was 3.9 years (41 days to 6.7 years). At last follow-up, one patient had undergone surgical conduit replacement for recurrent conduit stenosis, one had percutaneous pulmonary valve dilatation and two had developed bacterial endocarditis and undergone surgical conduit replacement. Freedom from valve dysfunction at 1, 2 and 3 years was 91%, 91% and 82%, respectively. CONCLUSIONS: PPVI is feasible in small conduits ≤ 16mm with good procedural and early haemodynamic results, postponing the need for surgery. These results are not sustainable over time. Larger studies are needed to determine if bare-metal stent implantation as a bridge to surgical replacement provides the same results in this specific population.

Neonatal management and outcomes of prenatally diagnosed CHDs.

OBJECTIVES: The aim of this study was to determine the probability of intervention at birth after prenatal diagnosis of CHD. METHODS: A 10-year retrospective study including all foetuses with a prenatally diagnosed CHD and those delivered in a tertiary-care cardiac centre between January, 2002 and December, 2011 was carried out. Patients were classified into eight groups according to the anticipated risk of neonatal intervention. RESULTS: The need for urgent intervention and/or PGE1 infusion within the first 48 hours of life was 47% (n=507/1080): 72% (n=248) for CHD at risk for a Rashkind procedure, 77% (n=72) for CHD with ductal-dependent pulmonary flow, 13% (n=22) for CHD with potentially ductal-dependent pulmonary flow, 94% (n=62) for CHD with ductal-dependent systemic flow, 29% (n=88) for CHD with potentially ductal-dependant systemic flow, 50% (n=4) for total anomalous pulmonary venous connection, and 17% (n=1) for CHD with atrio-ventricular block. In all, 34% of the patients received PGE1 infusion and 21.4% underwent urgent catheter-based or surgical interventions; 10% of patients without anticipated risk (n=10) underwent an early intervention; 6.7% (n=73) of the patients died; and 55% (n=589) had an intervention before discharge from hospital. CONCLUSION: Half of the neonates with foetal CHD benefited from an urgent intervention or PGE1 infusion at birth. We recommend scheduled delivery and in utero transfer for transposition of the great arteries, double-outlet right ventricle with sub-pulmonary ventricular septal defect, total anomalous pulmonary venous connection, CHD with atrio-ventricular block with heart rate <50, all ductal-dependant lesions, and CHD with potentially ductal-dependant systemic flow.

Discordances Between Pre-Natal and Post-Natal Diagnoses of Congenital Heart Diseases and Impact on Care Strategies.

BACKGROUND: Pre-natal diagnosis of congenital heart disease (CHD) allows anticipation of urgent neonatal treatment and provides adequate information to the parents on cardiac outcomes. OBJECTIVES: This study sought to analyze the discordances between expert fetal cardiac diagnosis and final diagnosis of CHD and their impact on neonatal and long-term care strategies. METHODS: We included 1,258 neonates with a pre-natally diagnosed CHD and 189 fetopsies following termination of pregnancy at our tertiary center over a 10-year period. Pre-natal echocardiographic and final diagnoses were compared. RESULTS: For live births, we identified 368 (29.3%) discordances between pre- and post-natal diagnoses. The pre-natal diagnosis was different from the post-natal diagnosis in 36 cases (2.9%) and partially different with a major impact on neonatal treatment of the CHD in 97 cases (7.7%). In 235 cases (18.7%), the diagnosis was partially different with no impact on neonatal planned treatment. The discordances had a negative impact on late care strategy in 62 cases (4.9%): more complex CHD that was unsuitable for biventricular repair, leading to unplanned compassionate care, additional surgery or increase of the complexity level of the Aristotle score. A positive impact was found in 31 cases (2.5%): less complex CHD that allowed biventricular repair, fewer surgical procedures, or decrease of the complexity of the Aristotle score. For 275 patients (21.9%), there was no impact on late care strategy. Of the 872 terminations of pregnancy and intrauterine fetal deaths, 189 fetopsies were available: 16 (8.5%) different diagnoses, 27 (14.3%) major differences, and 60 (31.7%) minor differences. CONCLUSIONS: Correcting fetal cardiac diagnosis after birth can lead to significant changes in neonatal (10.6%) and late (7.4%) care strategies. Tools should be developed to try to improve the accuracy of pre-natal diagnosis of CHD. Clinicians should be cautious when predicting required treatment and outcomes during pre-natal counseling.

Associated genetic syndromes and extracardiac malformations strongly influence outcomes of fetuses with congenital heart diseases.

BACKGROUND: Congenital heart disease (CHD) is often associated with extracardiac malformations (ECMs) and genetic syndromes. AIMS: To determine the effect of cytogenetic anomalies and/or ECMs associated with CHD on parental decision to choose termination of pregnancy (TOP) or compassionate care (CC), as well as on the outcome of children born alive. METHODS: This 10-year retrospective study included all prenatally diagnosed cases of CHD in a single tertiary referral centre. RESULTS: From January 2002 to December 2011, 2036 consecutive cases of fetal CHD (798 TOPs and 1238 live births, including 59 with postnatal CC) were included. CHD was associated with a known cytogenetic anomaly in 9.8% of cases and a major ECM in 11.7% of cases. The proportion of prenatally identified associated cytogenetic anomalies was significantly lower in the live-birth group than in the TOP plus CC group (4.2% vs 17.5%; P<0.001); this was also true for ECMs (8.1% vs 16.7%; P<0.001). The mortality rate was higher in the group with an associated cytogenetic anomaly or ECM (29.1%) than in cases with isolated CHD; a 2.4-fold increase in the death rate was observed (95% confidence interval 1.34-4.38; P=0.003). These associations remained significant after multivariable analysis, including the severity of the CHD (uni- or biventricular physiology). CONCLUSION: Prenatal diagnosis of a known cytogenetic anomaly or major ECM strongly influences parental decision to choose TOP or postnatal CC. Genetic syndromes and ECMs are associated with a higher mortality rate, independent of the complexity of the CHD.