Anomalous Connection of the Left Pulmonary Vein to the Coronary Sinus With Intact Atrial Septum in a Young Woman

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Anomalous Connection of the Left Pulmonary Vein to the Coronary Sinus With Intact Atrial Septum in a Young Woman

Background: Left sided Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital abnormal cardiac defect. An intact atrial septum is more uncommon. To our knowledge, connection of the left pulmonary vein (LPV) to the coronary sinus (CS) with intact atrial septum was no previous reported.Case report: We report an 18-years-old woman who had this rare anomaly. She exhibited no obvious clinical symptoms. An echocardiogram revealed the primary diagnosis and this diagnosis confirmed during operation. This patient underwent a successful surgical repair. Artificial atrial septal defect (ASD) and coronary sinus orifice were inserted into left atrium by patch. postoperative recovery was recuperated.Conclusion: Given the high risk of developing Congestive heart failure, like many other centers, we advocate for intervention during the preschool age. Surgical techniques depend on the number and location of abnormal veins or veins.

Novel technique of repairing right partial anomalous pulmonary venous connection with intact atrial septum using in situ interatrial septum as a flap in a 68-year-old-woman: a case report

Background Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown. Case presentation This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic. Conclusions The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.

Novel technique of repairing right partial anomalous pulmonary venous connection with intact atrial septum using in situ interatrial septum as a flap in a 68-year-old-woman: a case report

Background: Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown.Case presentation: This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic.Conclusions: The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.

Risk stratification of patients with hypoplastic left heart syndrome and intact atrial septum using fetal MRI and echocardiography

Despite prenatal diagnosis, prenatal intervention, and immediate postnatal intervention, patients with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have the highest risk for mortality. Charts for all infants diagnosed with hypoplastic left heart syndrome from 2009 to 2017 were retrospectively reviewed and compared, including pulmonary vein Doppler patterns on fetal echocardiogram and evidence of pulmonary lymphangiectasia on fetal MRI. Of the 81 newborns with hypoplastic left heart syndrome, we defined two groups. Group 1 patients had an adequate atrial septal communication (n = 69), while Group 2 met criteria for intact/restrictive septum (n = 12). No patient in Group 1 had a type C pulmonary vein Doppler pattern, while no patient in Group 2 had a type A pulmonary vein Doppler pattern. The two patients with pulmonary lymphangiectasia had type C pulmonary vein Doppler pattern and an intact atrial septum and did not survive. Survival to discharge for Group 1 was 83% compared to 58% for Group 2 (p = 0.116). Survival to stage 2 palliation was 71% for Group 1 compared to 50% for Group 2 (p = 0.186). Only 4 of the initial 12 patients from Group 2 are alive, which is an overall survival of 33%. Our experience supports previous evidence that fetal echocardiography can identify those patients with the greatest likelihood for postnatal intervention as well as those at highest risk for mortality. Fetal MRI is a novel imaging modality that may help providers separate patients at highest risk for mortality, regardless of pulmonary vein Doppler pattern.

P1729 Abnomral course of pulmonary vein causing right heart dilatation

Funding Acknowledgements None Introduction Partial anomalous pulmonary venous return is a rare congenital cardiac anomaly that usually involves the right pulmonary vein and an atrial septal defect. It is accounted for 0.7%. Isolated partial anomalous pulmonary venous return with an intact atrial septum is even rarer, and this condition is usually treated surgically in younger patients. We report a case of a young female, incidentally found to have a right upper pulmonary vein draining into the right atrium, confirmed by TOE and CT Venography pulmonary, with moderate right ventricular enlargement, without pulmonary hypertension, intra-cardiac shunt or accompanied congenital anomaly. A decision of surgical correction was made. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, even in absence of ASD. Case presentation A 26-year-old female, not known to have any chronic illness. She was referred because of poor appetite and low weight, to cardiology evaluation for accidental murmur finding during examination. She had no other symptoms. The TOE was done which showed left pulmonary veins are normal, the right lower pulmonary vein is normal, and there is high suspicion of aberrant right upper pulmonary vein draining in the right atrium, no evidence of intra-cardiac shunt. CT Venography pulmonary revealed anomalous (Giant) right superior pulmonary vein to the superior vena cava and draining to the right atrium. Figure 1. Patient was referred for surgical correction. Discussion Partial anomalous pulmonary venous return is a type of left to right shunt. It is a rare congenital abnormal cardiac defect in which the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Its accounted for 0.4 to 0.7%. However, as this rate is based upon autopsy data, the true prevalence of PAPVR may actually be higher. PAPVR is frequently associated with other congenital heart disease, most commonly ASD, in >75% of patients. It is estimated that 10% to 15% of patients with ASD and up to 85% of patients with sinus venosus ASD have PAPVC. Exclusive of ASD, other more complex cardiac malformations occur in ∼20% of patients. PAPVR can also be an isolated defect with intact atrial septum. TOE is more sensitive than TTE in detecting PAPVR. A potential limitation of echocardiography is the availability of acoustic windows. If all four PVs are not identified emptying into the left atrium on TEE, CCT and CMR may provide complementary data . CONCLUSION In conclusion, missed diagnosis always occurs in PAPVR, the debauched and blood flow of pulmonary vein should be over-viewed carefully during TEE examination. In a patient with unexplained RV enlargement PAPVC (one or more pulmonary veins) should be considered in the differential diagnosis. Multi-modality cardiac imaging using echocardiography, CT angiography and CMR may provide a comprehensive noninvasive evaluation of PAPVC. Abstract P1729 Figure. Fig1 TOE (Top),CT (Bottom) of RUPV