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Pulmonary venous hypertension may allow delayed palliation of single ventricle physiology with pulmonary hypertension


Department of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Center, Kochi, Kerala, India

Correspondence Address:
Atul Kalantre
Department of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Centre, Kochi - 682 041, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.177517

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Year : 2016  |  Volume : 9  |  Issue : 2  |  Page : 147-152

 

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Pulmonary vascular disease develops early in untreated single ventricle patients with increased pulmonary flow. Pulmonary artery (PA) banding is done at a young age in these patients in order to protect the lung vasculature and maintain low pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). This also enables future completion of the single ventricle palliation. Pulmonary venous hypertension (PVH) secondary to left sided obstruction if present in addition in this setting contributes to the pulmonary arterial hypertension (PAH) but involves an element of reversibility of the PAH if the obstruction is relieved. We present two cases of single ventricle both of who re-presented late with PAH and PVH (secondary to mitral valve obstruction) and underwent delayed PA banding at 9.5 and 4.5 years of age respectively. Both patients however had different outcomes. The patient undergoing PA banding at 9.5 years successfully underwent a cavo-pulmonary shunt at the age of 12 years. The patient with PA banding at 4.5 years however, has residual PAH that presently precludes a cavo-pulmonary shunt.






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Department of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Center, Kochi, Kerala, India

Correspondence Address:
Atul Kalantre
Department of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Centre, Kochi - 682 041, Kerala
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.177517

Rights and Permissions

Pulmonary vascular disease develops early in untreated single ventricle patients with increased pulmonary flow. Pulmonary artery (PA) banding is done at a young age in these patients in order to protect the lung vasculature and maintain low pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). This also enables future completion of the single ventricle palliation. Pulmonary venous hypertension (PVH) secondary to left sided obstruction if present in addition in this setting contributes to the pulmonary arterial hypertension (PAH) but involves an element of reversibility of the PAH if the obstruction is relieved. We present two cases of single ventricle both of who re-presented late with PAH and PVH (secondary to mitral valve obstruction) and underwent delayed PA banding at 9.5 and 4.5 years of age respectively. Both patients however had different outcomes. The patient undergoing PA banding at 9.5 years successfully underwent a cavo-pulmonary shunt at the age of 12 years. The patient with PA banding at 4.5 years however, has residual PAH that presently precludes a cavo-pulmonary shunt.






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