Of the 17 patients with CDH and CHD, 12 were male (71%). A total of 16 patients (94%) were diagnosed at the fetus stage. Thirteen patients had all date of CDH severity but four patients have some missing values and their severity of CDH was not available. The median gestational age was 37 (range 31–38) weeks, and the median body weight at birth was 2167 (range 1286–2822) g. Five patients had chromosomal abnormalities or genetic syndromes, including two with trisomy 13, one with trisomy 18, one with Goldenhar syndrome, and one with 15q11.2 micro deletion syndrome. CDH patients were primarily classified as left-sided (88%) or right-sided. All the patients underwent CDH repair. Patients with two ventricles were diagnosed with the following congenital heart defects: patent ductus arteriosus (n = 4), coactation of the aorta (n = 3), tetralogy of Fallot (n = 3), double-outlet right ventricle (n = 2), ventricle septal defect (n = 1), or complete transposition of the great arteries (n = 1). Three patients (18%) were diagnosed with a functional single ventricle (Table 1).
Table 1 showed the comparison between Group A and Group D. No significant differences were noted in gestational age, body weight at birth and chromosomal abnormality. There was no significant difference in the CDH severity between the two groups.
In Group A (n = 8), all patients had two ventricles and underwent definitive repair for congenital heart disease (Table 2). In Group D (n = 9), six patients had two ventricles and three patients had a single ventricle. Of the six patients with two ventricles, two patients underwent definitive repair. These two patients underwent tracheostomy and were discharged from the hospital, but later died due to airway obstruction at home, one surviving for 202 and the other for 3437 days, respectively. The other four patients who had two ventricles, including three patients with trisomy 13 or 18, did not undergo any cardiac surgery or intervention. All three patients with a functional single ventricle patient were in Group D. Two of these patients with a functional single ventricle underwent palliative cardiac surgery or intervention, but were not achieved with the Glenn procedure. The other patient died shortly after CDH repair and did not undergo cardiac surgery.
The details of the patient with two ventricles are shown in Table 4. Two patients (Cases 1 and 2) in Group A and two patients (Cases 9 and 10) in Group D who were required mechanical ventilation had high risk CDH. Cases 9 and 10 were discharged from the hospital and died at home due to airway obstruction. Four other patients died because of respiratory failure, intestinal perforation, cerebrovascular hemorrhage, and unknown causes. There were no cardiac-related deaths in group of patients with two ventricles.
The details of the three patients with a functional single ventricle are given in Table 4. The CDH was low risk in case 15. This patient underwent bilateral pulmonary artery banding and stent placement to repair patent ductus arteriosus. A pulmonary venous wedge pressure of 17 mmHg showed that there was no pulmonary hypertension. However, the patient required mechanical ventilation due to chronic respiratory dysfunction complicated by pulmonary artery hypoplasia and low cardiac function. Treatment for heart failure was prolonged, and sedation was always required. Although the Glenn procedure and other cardiac operations are considered to be at high risk surgical procedures, the patient survived for 560 days and died due to chronic respiratory function.
Case 16
who was diagnosed with a functional single ventricle whose CDH was high risk. After CDH repair, the patient underwent balloon atrio-septostomy and stent placement to correct a restrictive foramen ovale. The patient required mechanical ventilation due to tracheomalacia, and had repeated bouts of pneumonia that could not be controlled. After surviving 362 days post-surgery, the patient died due to refractory pneumonia.
Case 17
underwent CDH repair at the age of 1 day. Chest radiography revealed pleural effusion after the operation and the patient died due to sudden cardiac arrest immediately after drainage of the pleural effusion. The cause of cardiac arrest was unknown.