While on his community service, my friend Dr. VÃctor 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 VÃctor’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. VÃctor Hugo Roa-Castro for the case.
I have a beautiful 13 year old daughter. At the age of 6 months she developed a common cold. At the visit with my GP, we realised that something was horribly wrong. After a few weeks of consultation with peadiatric cardiologists she was diagnosed with Fallot’s Tetrology. At the age of 18 months we realised that she was in need of surgery. Today, she is a healthy 13 year old, a teenager who never misses what life has to offer her. She has an advanced brown belt in Karate and she excels academically. She is currently the deputy head girl of Milton Primary School, Three Rivers, Vereeniging, South Africa. I call her my miracle baby and to all parents who have children with the same condition I want to say that prayer, faith in God and the support of family and friends was what pulled us through. My daughter hopes to become a cardiologist one day. May God bless all our children.
I remember a case we had a few years ago in which a young pregnant patient, recently immigrated from somewhere in Central America and was diagnosed with Tetrology during her pregnancy! The kicker was, she couldn’t give consent to have surgery herself, as a minor. Her parents were still in another country, and refused! Wish I knew how it all turned out.
Imagine not catching it until she was 8! ToF seems so much more unpredictable ( and therefore dangerous) than HLHS. Or perhaps Im just hoping.