European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery | 2019
Nakata index above 1500\u2009mm2/m2 predicts death in absent pulmonary valve syndrome.
Abstract
OBJECTIVES\nAbsent 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\u2009days) and a threshold PA size for these outcomes.\n\n\nMETHODS\nThis retrospective 2-centre cohort study included 68 patients with complete repair between January 1996 and December 2015.\n\n\nRESULTS\nMedian age at repair was 3.9\u2009months (1.3-8.7\u2009months), and median weight was 5\u2009kg (4-7\u2009kg). 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\u2009<\u20090.001] and lower SpO2 (HR 1.06, 95% CI 1.02-1.09; P\u2009=\u20090.002). The accuracy of the Nakata index to predict death was excellent (area under the curve at 6\u2009months: 0.92; P\u2009=\u20090.010). A Nakata index above 1500\u2009mm2/m2 predicted mortality at 6\u2009months 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\u2009=\u20090.008). Neither PA plasty nor the LeCompte manoeuvre had a protective effect on mortality or prolonged ventilation. A Nakata index above 1500\u2009mm2/m2 remained a risk factor for mortality (P\u2009=\u20090.022) in patients who had a PA plasty or the LeCompte manoeuvre.\n\n\nCONCLUSIONS\nIn patients with absent pulmonary valve syndrome, the Nakata index predicts mortality with a cut-off of 1500\u2009mm2/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.