Polysplenia Syndrome as a Risk Factor for Early Progression of Pulmonary Hypertension
Akimichi Shibata1, Hiroki Mori2, Kazuki Kodo1
1Division of Pediatric Cardiology, Department of Pediatrics, Keio University School of Medicine.
Insights
Polysplenia syndrome, a condition affecting spleen development, is linked to higher pulmonary artery pressure in children with congenital heart disease. Early intervention is crucial to manage pulmonary hypertension in these patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Pulmonary Hypertension
Background:
- Advances in surgical and intensive care have improved outcomes for congenital heart disease (CHD) with heterotaxy syndrome.
- Pulmonary vascular complications in these patients remain less understood.
Purpose of the Study:
- To investigate pulmonary vascular complications in patients with polysplenia syndrome and congenital heart disease.
- To compare pulmonary hemodynamics in patients with and without polysplenia syndrome.
Main Methods:
- Retrospective review of 236 patients diagnosed with polysplenia syndrome.
- Comparison of 16 patients with polysplenia and incomplete atrioventricular septal defect (AVSD) against 22 age-matched controls with incomplete AVSD.
- Analysis of pulmonary artery pressure and vascular resistance before surgical intervention.
Main Results:
- Patients with polysplenia and incomplete AVSD exhibited significantly higher mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance index (PVRI) compared to controls.
- Polysplenia syndrome was identified as an independent risk factor for developing pulmonary hypertension (PH) in incomplete AVSD patients.
- The severity of systemic to pulmonary shunt did not significantly differ between groups.
Conclusions:
- Polysplenia syndrome is an independent risk factor for CHD-associated pulmonary hypertension.
- Earlier intervention may be necessary to manage pulmonary blood flow and prevent PH progression in polysplenia syndrome with CHD.
Background:
Recent progress in surgical and intensive care has improved the prognosis of congenital heart disease (CHD) associated with heterotaxy syndrome. Less is known, however, about pulmonary vascular complications in these patients.
Methods And Results:
We reviewed medical records of 236 patients who were diagnosed with polysplenia syndrome at 2 institutions for pediatric cardiology in Japan from 1978 to 2015. We selected and compared the clinical records of 16 patients with polysplenia who had incomplete atrioventricular septal defect (AVSD) as the polysplenia group, and 22 age-matched patients with incomplete AVSD without any syndromes including polysplenia as the control group. Although the severity of systemic to pulmonary shunt was not significantly different between the groups, mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance index (PVRI) were significantly higher in the polysplenia group than the control (mPAP, 37.3 vs. 19.1 mmHg, P=0.001; PVRI, 5.7 vs. 1.4 WU∙m2, P=0.014) before surgical intervention. On regression analysis, polysplenia influenced the development of pulmonary hypertension (PH) regardless of age at evaluation or degree of systemic to pulmonary shunt in the patients with incomplete AVSD.
Conclusions:
Polysplenia syndrome is an independent risk factor for CHD-associated PH. Earlier intervention may be required to adjust the pulmonary blood flow in polysplenia syndrome with CHD to avoid the progression of PH.
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