Definition.
Pulmonary venous ectopic drainage is a cardiac malformation in which the pulmonary veins are not directly connected to the left atrium but to the corpora cavernosa or right atrium. If all four pulmonary veins are abnormally connected to the body vein or right atrium, it is called “complete pulmonary vein ectopic drainage”, and most of them are combined with atrial septal defect or foramen ovale non-occlusion; if 1~3 of them are abnormally connected to the body vein or right atrium, it is called “partial pulmonary vein ectopic drainage”. “The severity of the lesion depends on the number of ectopic pulmonary vein branches, the size of the left-to-right shunt, the presence of atrial shunts and the obstruction of the ectopic pulmonary vein.
Morbidity.
Complete pulmonary venous ectopic drainage is an uncommon congenital cardiac anomaly, accounting for 1.5% of births with congenital heart disease. Partial pulmonary venous ectopic drainage accounts for approximately 0.67% of births with congenital heart disease.
Clinical presentation.
Complete pulmonary venous ectopic drainage: The child can have obvious clinical symptoms within one month after birth, such as shortness of breath and shortness of breath due to congestive heart failure, obvious cyanosis when crying or severe cyanosis when quiet, and some children have frequent episodes of hypoxia. The severity of symptoms in neonates and infants depends on the combination of pulmonary venous return obstruction and the degree of obstruction. In children and adults, the symptoms include frequent colds, cough, fever, post-activity panic, shortness of breath, sometimes palpitations, mild or severe cyanosis, and, in a few cases, hemoptysis or squatting. The clinical symptoms vary depending on the combined intracardiac malformation.
Partial pulmonary vein ectopic drainage: When single partial pulmonary vein ectopic drainage is not combined with other cardiac malformations, there are often no obvious symptoms. When multiple partial pulmonary vein ectopic drainage or (and) combined with atrial septal defect and other cardiac malformations, depending on the size of the left-to-right shunt flow, it can also be asymptomatic in infancy and childhood, and symptoms of heart failure, such as dyspnea, can also appear. Patients who also have a right-to-left shunt may present with symptoms such as cyanosis.
Diagnosis.
Diagnosis can be confirmed by physical signs, chest radiographs, electrocardiograms, cardiac color ultrasound, right heart catheterization and cardiac angiography, high-speed CT and MRI adherence.
Natural course.
Complete pulmonary venous ectopic drainage is a severe form of cyanotic congenital heart disease in which only 20% of children survive the first year of life. Most deaths occur in the first weeks and months of life, with approximately 50% dying within 3 months. Most children have respiratory distress, cyanosis and heart failure. Children who survive more than one year after birth without surgical treatment often have large atrial septal defects, while pulmonary vein obstruction, long pulmonary vein pathways and small patent foramen ovale are important causes of death in infants and young children. With increasing age, the resistance to pulmonary circulation gradually increases in some patients, resulting in severe pulmonary hypertension, and a few patients are eventually lost to surgery. Patients with partial pulmonary venous ectopic drainage have a milder condition than complete pulmonary venous ectopic drainage in the early stages, but the long-term presence of a larger amount of left-to-right shunt can gradually lead to the formation of pulmonary hypertension and thus affect survival.
Treatment: Once diagnosed, most patients require surgery.
Complete pulmonary venous ectopic drainage.
(i) Indications Early surgery is desirable. For neonates and infants with severe cyanosis, hypoxic episodes and heart failure, emergency surgery is required. In most children, surgery should be performed as soon as the diagnosis is confirmed. In some patients with mild symptoms, no obstruction of pulmonary venous return and large atrial septal traffic, surgery can be postponed until after 1 year of age.
(ii) Contraindications Severe pulmonary hypertension combined with Eisenmenger syndrome.
Partial pulmonary venous ectopic drainage.
(I) Indications The majority of PAPVD are combined with other cardiac malformations that require surgical treatment.
(ii) Contraindications Severe pulmonary hypertension combined with Eisenmenger’s syndrome.
Surgical complications.
Early postoperative complications: pulmonary edema, pulmonary hypertensive crisis, arrhythmias and pulmonary complications.
Late postoperative complications: mainly pulmonary venous obstruction and anastomotic stenosis and are the main cause of postoperative death and reoperation. There are also recurrent arrhythmias.
Surgical outcome.
Complete pulmonary vein ectopic drainage.
Early surgical outcomes for complete pulmonary vein ectopic drainage were not ideal, but surgical mortality has decreased significantly in recent years, although early postoperative mortality is significantly higher in patients with severe perioperative pulmonary hypertension.
Partial pulmonary vein ectopic drainage.
Partial type pulmonary vein ectopic drainage: has a good surgical outcome with an early postoperative mortality rate of 0-4%. There is no significant difference in early postoperative mortality between partial pulmonary venous ectopic drainage alone and atrial septal defect; the different anatomic types of partial pulmonary venous ectopic drainage are not a risk factor for operative mortality; the severity and severity of pulmonary vascular disease are important factors affecting early postoperative mortality and long-term postoperative outcome.