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Case History :

A 3 year-old girl presented with history of heart murmur noted in infancy. The child had restricted activities but normal milestones. Cardiologist's opinion was sought at the age of 2 years, when an Echo was done and a provisional diagnosis of small ASD was made, and the child was advised to wait some years for surgery.
On examination : The child is acyanotic, not dyspneic on routine activities, there was biventricular hypertrophy with loud P2, normal split and short systolic murmur in left 2nd interspace.
Echo : RVH, LVH, TR+. Calculated PA pressure>90mm Hg. Large PDA draining into MPA, with very little turbalance. AO PA pressure gradient 12 mmHg.

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Investigation Reports :

Click on the link below to view the Image/Movie and Description

Discussion :

When there is PAH in the presence of a large PDA there may be little or no diastolic component of the murmur, leading to mistaken diagnoses. Even on Echo, when there is little or no gradient across the PDA in the presence of Pulmonary hypertension, features of PAH with RV dilatation can lead to a wrong diagnosis of ASD. But the presence of LVH & disproportionate pulmonary hypertension in the presence of only a small ASD (most probably a PFO) should alert the cardiologist to the presence of shunt lesions or other pathology.
This child also has left lower lobe pulmonary vein obstruction which contributed to the development of early PAH.

Treatment options :
1.Surgical ligation of PDA + correction of pulmonary venous obstruction - morbidity is high in the presence of PAH.
2. Balloon dilatation of Pulmonary vein + Device closure of PDA.

Dr. Rama Bala,
Usha Mullapudi Cardiac Center