Unbounded Medicine

While on his community service, my friend Dr. Víctor Hugo Roa-Castro, just diagnosed a girl with Fallot’s Tetralogy (a congenital heart disease), wich consists in the combination of a ventricular septal defect (VSD) with Pulmonary Stenosis, with the Aorta “Overriding” (sitting “astride”) the VSD and with right ventricle hypertrophy (RVH).

It’s about 10% of all types of congenital heart disease. Both sexes affected with slight male predominance. Detected weeks or months after birth. Clinical features are cyanosis from birth or developing in the first year of life caused by the lung’s inability to oxygenate sufficient blood for the system, difficulty in feeding, failure to gain weight, retarded growth and physical development, dyspnea on exertion, clubbing of the fingers and toes, and polycythemia.

Here is the clinical case:

A “previously healthy” 8 years old came to Víctor’s consult because she has been feelling with fatigue in the last 7 months and her mother states that she tought that her daughter wasn’t sick despite her blueish tones in her skin since she was born.

On the clinical exam, Victor found this:

Cyanosis in the perioral area

Central cyanosis

Central cyanosis

Cyanosis & clubbing of the fingers

Cyanosis & clubbing of the fingers

Click on the player to hear the murmur

wich was a 3rd grade systolic in the 3rd left intercostal space.

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On the EKG: aQRS 150 grades, right ventricle hypertrophy, right atrial enlargement, transition V2-V3.

On the Chest X-ray: Situs solitus, cardio-thoracic index 0.6. Boot shaped heart with an upturned apex and a concave main pulmonary artery segment. Pulmonary flow appear slightly decreased.

On the Echocardiogram: Interventricular abnormal communication (restrictive type), Aorta overriding, right ventricular hypertrophy, infundibular pulmonary stenosis.

Many thanks to Dr. Víctor Hugo Roa-Castro for the case.