Pediatric case # 3

Splittgerber FH, Schmaltz AA (Essen Univ. Med. Center, Pediatric Cardiology)

10 year old girl who had been hospitalized a number of times after she was originally diagnosed to have a "dilated cardiomyopathy" at the age of 6 months. Recent echocardiograms were interpreted as showing a "hypertrophic cardiomyopathy". Unusually large coronary arteries were visualized on echo in the interventricular septum. Cardiac catheterization was performed to demonstrate any coronary artery anomaly.
Location Pressure mmHg (mmHg) Saturation (%)
RA 10/7/6 high RA  70
low RA   76
RV 54/2/12 77
PA 57/12/27 80
LV 132/-3/19 99
Ao asc. 125/75/98 98
Ao desc. 116/73/94 99
The calculated Qp/Qs was 1.3.

Supravalvular aortography is diagnostic:

Supravalvular aortography - early phase pediatric case #3Very large RCA, no LCA visible.

supravalvular aortography - pediatric case #3Retrograde filling of the left coronary system; PA opacifies.

Dx: ALCAPA (Bland-White-Garland syndrome)

supravalvular aortography - late phase ped. case #3Opacification of the PA via the LCA. This clearly demonstrates the underlying pathophysiology as observed after the age of about 2 months: retrograde flow from the left coronary artery system into the PA - as opposed to mixed venous blood flowing into the LCA from the PA.
 

This child underwent successful surgery: translocation of the anomalous LCA into the aorta. The anomalous takeoff was from the right non facing PA sinus. LCA mobilization made it possible to anastomose the vessel with a button of surrounding tissue close to a normal location at the left aortic sinus. The defect in PA sinus was repaired with a pericardial patch. The LCA origin had been close to a pulmonary valve commissure which was left undisturbed. The child recovered without problems. 
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Copyright Fred Splittgerber.

last update Sept 21, 1999