Segmental Pulmonary Hypertension in Complex Congenital Heart Disease: The Role of Multimodality Imaging.

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.