Abnormalities of the circulatory system
Congenital heart defects are abnormalities of the heart that occur during its development in the embryonic period. They are among the most common congenital anomalies in children (~1% of births). They vary widely in severity, ranging from benign abnormalities to severe malformations that are incompatible with the newborn’s survival.
Aortic coarctation
Aortic coarctation is a congenital narrowing of the aorta located at the exit of the left ventricle. The narrowing can vary in severity, even extending to an interruption of the aortic arch. This anomaly occurs more frequently in boys and leads to left ventricular hypertrophy. In most cases, it is accompanied by heart disease. Diagnosis may be delayed in a patient presenting with a combination of a loud systolic murmur, absent femoral pulses, and high blood pressure.
Register |
Period |
Births |
Live births |
Stillbirths (excluding IMG) |
IMG |
Total |
Total prevalence/10,000 (95% CI) |
Antilles |
2011–2015 |
47,493 |
14 |
14 |
2.9 (1.6–4.9) |
||
Auvergne |
2011–2015 |
66,381 |
23 |
3 |
26 |
3.9 (2.6–5.7) |
|
Brittany |
2011–2015 |
179 180 |
60 |
1 |
8 |
69 |
3.9 (3.0–4.9) |
Paris |
2011–2015 |
128,915 |
34 |
1 |
35 |
2.7 (1.9–3.8) |
|
Reunion |
2011–2015 |
71,756 |
23 |
23 |
3.2 (2.0–4.8) |
||
Rhône-Alpes |
2011–2015 |
292,693 |
108 |
2 |
10 |
120 |
4.1 (3.4–4.9) |
Source: French Registries of Congenital Anomalies, INSEE for births in 2011–2015
Transposition of the Great Arteries
Transposition of the great arteries is a heart defect characterized by the reversed position of the vessels at the base of the heart: the pulmonary artery originates from the left ventricle and the aorta originates from the right ventricle, unlike in a normal heart.
Register |
Period |
Births |
Live births |
Stillbirths (excluding IMG) |
IMG |
Total |
Total prevalence/10,000 (95% CI) |
Antilles |
2011–2015 |
47,493 |
11 |
2 |
4 |
17 |
3.6 (2.1–5.7) |
Auvergne |
2011–2015 |
66,381 |
8 |
9 |
17 |
2.6 (1.5–4.1) |
|
Brittany |
2011–2015 |
179 180 |
56 |
10 |
66 |
3.7 (2.8–4.7) |
|
Paris |
2011–2015 |
128,915 |
34 |
2 |
6 |
42 |
3.3 (2.3–4.4) |
Reunion |
2011–2015 |
71,756 |
26 |
1 |
2 |
29 |
4.0 (2.7–5.8) |
Rhône-Alpes |
2011–2015 |
292,693 |
158 |
2 |
37 |
197 |
6.7 (5.8–7.7) |
Source: French Registries of Congenital Anomalies, INSEE for births in 2011–2015
For more information: Orphanet (transposition of the great arteries)
Tetralogy of Fallot
Tetralogy of Fallot is characterized by a ventricular septal defect, an obstruction of the right ventricular outflow tract, an aortic root "straddling" the ventricular septum, and right ventricular hypertrophy.
Register |
Period |
Births |
Live births |
Stillbirths (excluding IMG) |
IMG |
Total |
Total prevalence/10,000 (95% CI) |
Antilles |
2011–2015 |
47,493 |
6 |
2 |
8 |
1.7 (0.7–3.3) |
|
Auvergne |
2011–2015 |
66,381 |
17 |
2 |
4 |
23 |
3.5 (2.2–5.2) |
Brittany |
2011–2015 |
179 180 |
55 |
1 |
40 |
96 |
5.4 (4.3–6.5) |
Paris |
2011–2015 |
128,915 |
31 |
2 |
8 |
41 |
3.3 (2.3–4.4) |
Reunion |
2011–2015 |
71,756 |
15 |
1 |
2 |
18 |
2.5 (1.5–4.0) |
Rhône-Alpes |
2011–2015 |
292,693 |
121 |
3 |
36 |
160 |
5.5 (4.7–6.4) |
Source: French Registries of Congenital Anomalies, INSEE for births in 2011–2015
For more information: Orphanet (Tetralogy of Fallot)
Left Ventricular Hypoplasia
Left heart hypoplasia syndrome (LHHS) is one of the most severe congenital heart defects. It refers to a developmental abnormality of the left-sided structures of the heart, obstructing blood flow in the vessels originating from the left ventricle. This syndrome is also characterized by insufficient development of the left ventricle, the aorta, and the aortic arch, as well as mitral atresia or stenosis.
Register |
Period |
Births |
Live births |
Stillbirths (excluding IMG) |
IMG |
Total |
Total prevalence/10,000 (95% CI) |
Antilles |
2011–2015 |
47,493 |
5 |
12 |
17 |
3.6 (2.1–5.7) |
|
Auvergne |
2011–2015 |
66,381 |
7 |
1 |
23 |
31 |
4.7 (3.2–6.6) |
Brittany |
2011–2015 |
179 180 |
15 |
4 |
36 |
55 |
3.1 (2.3–4.0) |
Paris |
2011–2015 |
128,915 |
12 |
24 |
36 |
2.8 (2.0–3.9) |
|
Reunion |
2011–2015 |
71,756 |
11 |
5 |
14 |
30 |
4.2 (2.8–6.0) |
Rhône-Alpes |
2011–2015 |
292,693 |
39 |
5 |
85 |
129 |
4.4 (3.7–5.2) |
