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Association of interrupted aortic arch with malformations producing reduced blood flow to the fourth aortic arches
Insights
Interrupted aortic arch, a congenital heart defect, may result from blood flow imbalances during early development, affecting the embryonic aortic arch system. This imbalance leads to a loss of aortic arch continuity, impacting blood flow to the descending aorta.
Area of Science:
- Cardiovascular Surgery
- Developmental Biology
- Pediatric Cardiology
Background:
- Interrupted aortic arch is a rare congenital malformation characterized by a discontinuity between the proximal and descending aorta.
- This condition typically requires a patent ductus arteriosus for survival, supplying blood to the descending aorta.
- Understanding the developmental origins is crucial for diagnosis and treatment.
Purpose of the Study:
- To investigate the anatomical patterns and potential developmental mechanisms of interrupted aortic arch.
- To categorize the outflow tract anomalies associated with interrupted aortic arch.
- To explore the relationship between embryonic aortic arch development and interrupted aortic arch.
Main Methods:
- Retrospective review of 12 autopsy cases of interrupted aortic arch at The Johns Hopkins Hospital.
- Detailed evaluation of heart specimens, focusing on outflow tract relations and associated malformations.
- Analysis of blood flow patterns through embryonic aortic arch remnants.
Main Results:
- Four distinct categories of interrupted aortic arch were identified based on outflow tract anomalies.
- Common findings included ventricular septal defects with pulmonary arterial override, mitral valve obstruction, double inlet left ventricle, and truncus arteriosus.
- A consistent pattern suggested preferential flow into the sixth aortic arches and reduced flow into the fourth arches.
Conclusions:
- Interrupted aortic arch may arise from the disappearance of the normal connection between the left fourth and sixth aortic arches.
- This developmental anomaly is likely caused by an imbalance in embryonic blood flow during early cardiogenesis.
- The study provides insights into the embryological basis of interrupted aortic arch, aiding in understanding its complex etiology.
Abstract:
Interrupted aortic arch is a rare congenital malformation of the great arteries with loss of continuity between the proximal portion of the aortic arch and the descending aorta, which is usually supplied by a patent ductus arteriosus. The hearts of 12 patients with interrupted aortic arch listed in the autopsy files of The Johns Hopkins Hospital were evaluated. Four categories were identified. Seven hearts with normal outflow tract relations had significant pulmonary arterial override of a ventricular septal defect and thus recevived part of left ventricular and all of right ventricular ejection flow; flow into the aorta was correspondingly reduced. Two hearts had mitral valve obstruction with decreased left heart flow. Two hearts had double inlet left ventricle and transposed great arteries with the aorta originating from a small anterior conal chamber. One heart had a truncus malformation with a disproportionately large flow entering the pulmonary arterial division. In each of these hearts, the pattern of the outflow tracts and associated malformations suggested that there was a preferential flow of blood into the sixth arches and correspondingly a reduced flow into the fourth arches at the time of normal disappearance of components of the embryonic aortic arch system. Thus, interrupted aortic arch may simply be disappearance of the normally persisting connection between the left fourth and sixth arches as a result of flow imbalance in early cardiogenesis.