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Segmental Pulmonary Hypertension in Complex Congenital Heart Disease: The Role of Multimodality Imaging
Panayotis K Vlachakis1, Eva Nyktari2, Anastasios Apostolos1
1First Cardiology Department, Athens Medical School, Hippokration General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Background:
A 21-year-old male with complex congenital heart disease (pulmonary atresia with intact ventricular septum and major aortopulmonary collateral arteries [MAPCAs]) was seen in our outpatient clinic. Diagnosed shortly after birth, the patient underwent Rashkind atrial septostomy and was deemed amenable to surgical correction/partial unifocalization or palliative intervention.
Case Summary:
On examination, the patient was cyanotic with no signs of heart failure. Multimodality imaging showed MAPCAs from the aorta to the pulmonary circulation with a variable degree of stenosis suggestive of segmental pulmonary hypertension (PH). The decision of the multidisciplinary meeting was for a conservative approach and close monitoring with the potential of palliative interventions if there is a deterioration in patients' functional status.
Discussion:
Segmental PH presents a complex scenario, necessitating multimodality imaging for therapeutic considerations.
Take-Home Message:
In cyanotic congenital heart disease, the presence of MAPCAs and segmental PH presents a complex scenario, necessitating multimodality imaging.
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