Cardiovascular

Congenital Heart Disease Associated With Genetic Syndromes and Extracardiac Anomalies: A Six-Year Epidemiological Study in a Brazilian Referral Center.

TL;DR

Among 5647 births at a Brazilian tertiary referral center over six years, 225 newborns with congenital heart disease were identified (prevalence ~4%), with 64% having associated congenital anomalies and 42.2% confirmed genetic syndromes, and the presence of associated anomalies and syndromes negatively impacted neonatal prognosis.

Key Findings

The prevalence of congenital heart disease (CHD) among newborns at this Brazilian tertiary referral center was approximately 4%.

  • Among 5647 total births, 225 newborns with CHD were identified and analyzed.
  • This prevalence reflects the highly complex patient profile of a high fetal-risk maternity hospital.
  • The study period spanned January 2018 to December 2023 (six years).
  • Data were drawn from the ECLAMC (Latin American Collaborative Study of Congenital Malformations) database at Instituto Fernandes Figueira/Fiocruz (IFF/Fiocruz) in Rio de Janeiro.

Ventricular septal defect (VSD) was the most frequent CHD, followed by atrioventricular septal defect (AVSD) and coarctation of the aorta (CoA).

  • VSD was present in 68 cases.
  • AVSD was identified in 41 cases.
  • CoA was found in 27 cases.
  • Complex cardiopathies predominated overall, representing 60% of all CHD cases.

The majority of CHD diagnoses were established prenatally, with fetal echocardiogram demonstrating moderate accuracy.

  • 93.3% of diagnoses were established prenatally.
  • Fetal echocardiogram accuracy was 78.5%.
  • The study was conducted at a high fetal-risk maternity hospital, which may have contributed to the high rate of prenatal detection.

Associated congenital anomalies were identified in 64% of newborns with CHD, predominantly affecting the gastrointestinal tract, central nervous system, and genitourinary tract.

  • 64% of the 225 CHD cases had associated congenital anomalies.
  • The most commonly affected systems were the gastrointestinal tract, central nervous system, and genitourinary tract.
  • The high rate of associated anomalies reflects the tertiary referral nature of the center.

Genetic syndromes were confirmed in 42.2% of CHD patients, with Down syndrome being the most common, followed by Edwards syndrome and Patau syndrome.

  • Genetic syndromes were confirmed in 95 of 225 patients (42.2%).
  • Down syndrome was the most prevalent, identified in 35 patients.
  • Edwards syndrome was present in 29 patients.
  • Patau syndrome was identified in 10 patients.

The rate of hospital discharge alive was substantially lower in CHD patients with associated genetic syndromes or anomalies compared to those with isolated CHD.

  • The hospital discharge alive rate was 53.3% in patients with isolated CHD.
  • The hospital discharge alive rate was 29% in patients with associated syndromes or anomalies.
  • This difference highlights the negative impact of associated anomalies and syndromes on neonatal prognosis.
  • The authors note this finding reinforces the importance of prenatal diagnosis and specialized multidisciplinary care.

What This Means

This research examined six years of birth data (2018–2023) at a major referral hospital in Rio de Janeiro, Brazil, to understand how common heart defects present at birth are, what other health conditions they are associated with, and what happens to affected newborns. Out of nearly 5,700 births, about 1 in 25 newborns (4%) had some form of congenital heart disease (CHD). The most common type was a hole between the lower chambers of the heart (ventricular septal defect), and 60% of all heart defects were classified as complex. Encouragingly, over 93% of cases were detected before birth through prenatal screening, though the prenatal ultrasound of the heart (fetal echocardiogram) correctly identified the condition about 79% of the time. A striking finding was how frequently heart defects occurred alongside other health problems. Nearly two-thirds of affected newborns also had other structural abnormalities — most often affecting the digestive system, brain and nervous system, or urinary and reproductive organs. Additionally, more than 4 in 10 newborns with CHD had a confirmed genetic syndrome, most commonly Down syndrome (trisomy 21), Edwards syndrome (trisomy 18), or Patau syndrome (trisomy 13). These genetic conditions are known to significantly affect multiple organ systems and overall survival. This research suggests that the presence of additional congenital anomalies or genetic syndromes significantly worsens outcomes for newborns with heart defects. Babies with heart defects alone had a hospital survival rate of about 53%, while those with additional syndromes or anomalies survived to discharge only about 29% of the time. These findings highlight the importance of early prenatal detection and coordinated care from teams of specialists — including cardiologists, geneticists, and neonatologists — for families affected by congenital heart disease, particularly when other conditions are also present.

Have a question about this study?

Citation

Rodriguez L, Lerner N, Leite M, Farias C, Barmpas M, Orofino D, et al.. (2026). Congenital Heart Disease Associated With Genetic Syndromes and Extracardiac Anomalies: A Six-Year Epidemiological Study in a Brazilian Referral Center.. Birth defects research. https://doi.org/10.1002/bdr2.70111