Authored By: Zhongshan Gou from Dept. U.S.
Hospital Name: The Affiliated Suzhou Hospital of Nanjing Medical University
Instruments and Methods:
Patient History:
Complaint:
Punctate hyperechoic in fetal left ventricular(LV)
By now, everything is normal. The preliminary diagnosis suggested no significant abnormality in fetal heart structure at present. However, when the probe is slightly tilted from the standard four chamber view to the fetal foot side, the problem occurs:
Accordingly, it should be diagnosed as partial anomalous pulmonary venous return (PAPVR, Type II). Intracardiac: left superior pulmonary veins(LSPV) and Left inferior pulmonary vein(LIPV) return to coronary sinus (PAPVR to CS).
Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiovascular condition in which one or more (but not all) of the pulmonary veins drain into the right atrium or systemic circulation rather than in the left atrium.
According to the location of ectopic drainage, intracardiac, supracardiac and subcardiac are concluded. Intracardiac type is the most common type while subcardiac type is rare. Right-sided PAPVR is considered one of the most frequent types. And the most common direct drainage of right pulmonary vein is right atrium. Generally, the right pulmonary vein ectopic drainage often leads to the superior vena cava, inferior vena cava, right atrium, azygos vein, portal vein and hepatic vein. On the other hand, anomalous pulmonary venous drainage often leads to the left cephalic brachial vein, coronary sinus and hemiazygos vein.
One or more pulmonary venous blood is drained to the venous system of the systemic circulation, and part of the pulmonary venous blood is drained to the left atrium(LA), resulting in left-to-right shunting, pulmonary hypertension (PH), right heart insufficiency(CHF), and sinus node dysfunction would happen.
The severity of clinical symptoms depends on the degree of left to right shunt. Patients with small shunt flow may not have any clinical symptoms for life. While patients with multiple anomalous PVD often have chest pain, palpitation and dyspnea, and some patients may have severe PH.
In principle, surgical treatment have to be done for these children. Others believe that when a single anomalous pulmonary venous drainage (APVD) occurs, follow-up observation can be carried out as the small shunt volume and without other malformations. However, patients with more than 2 ectopic drains should be treated with early surgery because of large drainage volume, which is equivalent to large atrial septal defect.
Transposition suture of atrial septum, anastomosis of pulmonary vein and left atrium with ectopic drainage are used for surgical. Intra-atrial patch repair and vertical venous left atrial appendage anastomosis are also often used. Drainage of partial pulmonary venous malformations is effective as early postoperative mortality is less than 4%.
References:
1. Rong Jin, Yi Luo, Pei Cheng, et al. Diagnosis and treatment of partial anomalous pulmonary venous drainage. Chinese Journal of medicine, 2008, 88 (15): 1066-1068
2. Yoshihara K, Ozawa T, Sakuragawa H, et al. 1998. Surgical treatment of total anomalous pulmonary venous connections-clinical aspects of pulmonary venous obstruction. J. Tnorac. Cardiovasc Surg, 46:1126-1132.