Hypertrophic cardiomyopathy - neonatal

Case contributed by Priya Parikh

Presentation

Ex 37 weeker baby boy, infant of diabetic mother born via C-section with respiratory distress.

Patient Data

Age: 0 days
Gender: Male

1. Initial XR chest evaluation demonstrates moderately severe cardiomegaly with mild to moderate diffuse pulmonary vascular congestion and mild to moderate pulmonary interstitial edema.

2. ​There is slight asymmetric increased opacification of the left lung field likely due to dependent edema. ​

3.  No definite effusion or pneumothorax. No osseous anomaly.

4. The orogastric tube courses into the stomach.

1. ETT terminates at T1 level. OG tube courses into the stomach. UVC is seen terminating in the low right atrium.

2. Mild improved bilateral lung aeration. Now noted is increased diffuse haziness mainly in the left upper and midzone, most likely due to asymmetric pulmonary edema.

3. Stable moderately severe cardiomegaly and pulmonary interstitial edema.

4. No definite effusion or pneumothorax. No osseous anomaly.

Case Discussion

Full-term infant of a diabetic mother born via C-section with respiratory distress and a systolic murmur. Initial chest x-ray demonstrated moderately-severe cardiomegaly and pulmonary vascular congestion and interstitial edema. An echocardiogram was performed which confirmed marked biventricular hypertrophy in addition to other cardiac findings including hypertrophied interventricular septum (thickness measuring 9 mm), dilated coronary arteries and narrowed aortic isthmus.  

Infants of diabetic mothers are at increased risk for cardiac valvular and septal lesions in addition to hypertrophic cardiomyopathy. In a large retrospective study, the prevalence of cardiovascular malformations in infants of diabetic mothers approached 4%, compared to less than 1% in babies with non-diabetic mothers 1.

The incidence of symptomatic hypertrophic cardiomyopathy is reported to be as high as 12.1% of infants with diabetic mothers 2. Other associated findings include left ventricular outflow tract obstruction and reduced stroke volume 4. Septal enlargement and hypertrophy are secondary to fetal hyperinsulinemia.

This case was submitted with supervision and input from:

Soni C. Chawla, M.D.
Associate Professor
Department of Radiological Sciences
David Geffen School of Medicine at UCLA
Olive View - UCLA Medical Center  

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Case information

rID: 63314
Published: 28th Sep 2018
Last edited: 28th Sep 2018
Inclusion in quiz mode: Included
Institution: Olive View - UCLA Medical Center

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