A normal heart has two ventricles. In Mai's case, it was initially unclear whether her heart had one or two chambers, making her condition a complex defect between a single and double ventricle heart.

Mai's third surgery took place on 17/6 at the Cardiovascular Center of Tam Anh General Hospital in Ho Chi Minh City when she was 7 years old. Dr. Vu Nang Phuc, Head of the Congenital Heart Department, reported that upon admission, Mai showed cyanosis, with blood oxygen saturation (SpO2) dropping to 78%. Her pulmonary artery pressure and resistance were at the upper limit. Additionally, a large collateral circulation connecting the descending aorta to the pulmonary artery was overloading her lungs.

Doctors perform heart surgery on the patient. Illustrative photo: Huyen Vu

Mai's condition, pulmonary atresia with intact ventricular septum (PA/IVS), was detected during a prenatal ultrasound at 22 weeks.

Two days after birth, she underwent her first surgery at another hospital to widen the pulmonary valve opening and place a stent to temporarily restore blood flow to her lungs. For the first 8 months of her life, Mai received regular check-ups and echocardiograms. She then had a second surgery, a Glenn procedure (connecting the superior vena cava to the pulmonary artery), to reduce the strain on her heart and improve blood oxygen saturation. This improved her health, but her skin remained slightly bluish.

In the recent surgery, Dr. Phuc's team completely closed the collateral circulation and treated Mai's pulmonary artery dilation to reduce pressure and prepare for the major Fontan operation. The 6-hour surgery, performed by Dr. Nguyen Minh Tri Vien, Surgical Advisor, and Dr. Tran Thuc Khang, Deputy Head of Cardiothoracic Surgery, involved creating a graft to connect the inferior vena cava to the right pulmonary artery, with a small fenestration to temporarily reduce pressure.

Post-surgery, Mai's SpO₂ rose to 94-95%, and her central venous pressure remained below 15 mmHg. The endotracheal tube was removed 10 hours after the operation. There was no bleeding or arrhythmia. A week later, Mai was discharged with healthy pink lips and fingertips. She was prescribed anticoagulants and pulmonary vasodilators, along with regular follow-up appointments to monitor for long-term complications of the Fontan circulation.

Studies show that PA/IVS affects about 5 in 100,000 live births, making it a relatively rare congenital heart defect. According to Dr. Khang, most patients undergo a three-stage treatment plan. First, they receive neonatal intervention with valve dilation and possibly stent placement in the arterial duct to balance blood flow to the lungs. Between 6 and 12 months of age, they undergo the Glenn procedure to redirect blood from the upper body to the lungs, relieving the single ventricle. Between 3 and 7 years of age, the Fontan procedure redirects blood from the lower body to the lungs, completing the single-circulation pathway.

"Prerequisites for the success of these surgeries are low pulmonary artery pressure and resistance, absence of major collateral circulation, good ventricular function, and stable sinus rhythm," Dr. Khang explained. Dr. Phuc added that prenatal and neonatal echocardiograms are crucial for early detection of defects like PA/IVS. Timely intervention and surgery can enable children to live healthy lives, with physical activity levels reaching approximately 80%.

Parents should seek medical attention for their children if they exhibit symptoms such as blue lips and fingertips, rapid breathing, slow weight gain, or poor exercise tolerance. After Fontan surgery, lifelong monitoring is necessary to detect complications like graft obstruction, systemic venous hypertension, arrhythmia, and liver failure.

Thu Ha

*The patient's name has been changed