Congenital diaphragmatic hernia with congenital heart disease including ventricular septal defect: Risk factors.

BACKGROUND

Congenital diaphragmatic hernia (CDH) with congenital heart disease (CHD) has a poor prognosis. This study aimed to establish the prognostic factors of CDH associated with ventricular septal defect (VSD), which is the most commonly encountered CHD.

METHODS

We conducted a single-center, retrospective observational study including neonatal cases of CDH with CHD managed at our hospital between 2000 and 2022. The risk factors for CDH in patients with VSD were established by comparing survivors and non-survivors.

RESULTS

Among the 84 infants with CDH, 14 (17%) were associated with CHD, including 11 cases of VSD (79% of CHD). The survival rate of patients with CDH prenatally diagnosed with left-sided disease and VSD was 56% (four of nine patients died). The rate of low lung volume (observed/expected lung area-to-head circumference ratio ≤ 35%) was significantly higher in non-survivors than in survivors (75% vs. 0%, p = 0.048). All non-survivors had associated severe CHD (n = 3) or severe pulmonary hypertension (n = 1).

CONCLUSION

Low lung volume was a risk factor for CDH with at least moderate to small size of VSD, which was comparable with that in patients with isolated CDH.