Decreased Pulmonary Arterial Compliance is a Predictor for Poor Outcomes in Infants with Isolated Atrial Septal Defect a
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ORIGINAL ARTICLE
Decreased Pulmonary Arterial Compliance is a Predictor for Poor Outcomes in Infants with Isolated Atrial Septal Defect and Pulmonary Hypertension Yuki Iwaya1 · Jun Muneuchi1 · Mamie Watanabe1 · Yuichiro Sugitani1 · Yoshie Ochiai2 Received: 6 April 2020 / Accepted: 12 June 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract This study aimed to investigate risk factors for poor outcomes in infants with isolated atrial septal defect (ASD) and pulmonary hypertension who had unexpectedly fatal course. We retrospectively reviewed 22 infants with isolated ASD and pulmonary hypertension, and analyzed the relationship between clinical outcomes and pulmonary hemodynamic parameters including pulmonary arterial resistance (Rp) and compliance (Cp) based on cardiac catheterization among them. Age and weight at cardiac catheterization were 5 (1–11) months and 4.9 (3.1–9.2) kg, respectively. There were 17 individuals with Down syndrome. Pulmonary hemodynamic parameters were shown as follows: the ratio of pulmonary to systemic blood flow: 2.0 (0.6–3.8), mean pulmonary arterial pressure: 41 (20–60) mmHg, the ratio of pulmonary to systemic mean pressure (Pp/Ps): 0.67 (0.46–1.13), Rp: 4.11 (0.68–15.80) Wood units m2, and Cp: 1.80 (0.63–6.16) mL/mmHg m2. There were 4 deaths during the follow-up period of 40 (7–241) months. Univariable logistic regression analysis revealed that Pp/Ps (odds ratio [OR]: 18,500, 95% confidential interval [CI] 1.48–230,659,690, P = 0.041) and Cp (OR: 0.03, 95% CI 0.001–0.73, P = 0.031) were significantly related to death. A Pp/Ps cutoff value more than 0.94 and a Cp cutoff value less than 0.97 mL/mmHg m2 yielded as the predictors of death with sensitivity of 98% and 94%, specificity of 75% and 75%, respectively. Decreased Cp is a previously unrecognized predictor for poor outcome in infants with isolated ASD and pulmonary hypertension. Keywords Pulmonary arterial hypertension · Pulmonary arterial capacitance · Trisomy 21 · Congenital heart disease
Introduction Infants with isolated atrial septal defect (ASD), that is the stereotypical congenital heart disease, are usually less symptomatic, and corrective surgery is typically carried out beyond infancy. However, we occasionally encounter infants with isolated ASD and severe pulmonary hypertension who have postoperative comorbidities and unexpectedly fatal courses, especially associated with Down syndrome or premature born. [1–5] Although early corrective repair may be considered in such patients, they should be * Jun Muneuchi [email protected] 1
Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, 1‑8‑1, Kishinoura, Yahatanishi‑ku, Kitakyushu, Fukuoka 806‑8501, Japan
Department of Cardiovascular Surgery, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan
2
appropriately managed according to combined multidisciplinary approach because the development of pulmonary hypertension is caused by heterogenous factors including left-to-right shunt, abnor
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