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

Abstract

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.

Assessment of Intracardiac and Extracardiac Deformities in Patients with Various Types of Pulmonary Atresia by Dual-Source Computed Tomography

“…Different McGoon ratios and PAI indicate the development of the NPA situation. A higher ratio means a lower risk of surgery and a better outcome [21,22]. Thus, patients with low surgical indices could not undergo the one-stage surgery, which helps to explain the fact that more patients in the two latter groups did not undergo one-stage surgery in this study.…”

Right ventricular afterload in repaired D-TGA is associated with inefficient flow patterns, rather than stenosis alone

“…While the Nakata index was originally validated in Tetrology of Fallot as well as Rastelli and Fontan patients, it has been shown to be consistent across infants and adolescents [23]. Nakata index was utilized here as an indexed continuous variable of PA size without the confounder of BSA to evaluate relationships between PA dimensions and hemodynamic variables, similar to previous studies [24,25]. All measurements were performed by a blinded observer.…”