Isolated Partial Anomalous Pulmonary Veins: A 10-Year Experience at a Single Center.

INTRODUCTION: Isolated partial anomalous pulmonary venous connection (PAPVC) is difficult to diagnose, and surgical indications remain controversial. We reviewed 10 y of isolated PAPVC cases. METHODS: The data of patients with isolated PAPVC admitted to the Anzhen Congenital Heart Disease Department from 2010 to 2019 were reviewed retrospectively. RESULTS: Thirty patients, aged between 4 mo and 32 y, were included in this study. Significant correlations were found between the right ventricle (RV), end-diastolic dimension Z-score (RVED-z) and age (r = 0.398, P = 0.03), and between estimated pulmonary pressure and age (r = 0.423, P = 0.02). However, no significant correlations were found between the RVED-z and the number of anomalous pulmonary veins (r = 0.347, P = 0.061), between estimated pulmonary pressure and the RVED-z (r = 0.218, P = 0.248), and between estimated pulmonary pressure and the number of anomalous veins (r = 0.225, P = 0.232). Transthoracic echocardiography (TTE) confirmed 90% of isolated PAPVC cases. Surgical repair was performed in 29 patients with RV enlargement, persistent low weight, pulmonary hypertension, or respiratory symptoms. Among the surgical patients, nine had elevated pulmonary pressure before surgery, which decreased postoperatively; no mortality or reintervention was observed. The mean duration of echocardiographic follow-up was 1.9 y. CONCLUSIONS: TTE is recommended for routine assessments, and further clarification can be obtained with computed tomography when TTE proves inconclusive for diagnosis. Transesophageal echocardiography and computed tomography are further recommended for adult patients if TTE fails to provide clear results. PAPVC should be considered as an underlying cause when unexplained RV enlargement is observed. Surgery is recommended for patients with RV enlargement, pulmonary hypertension, or respiratory symptoms.

Video assisted thorascopic assisted correction of left partial anomalous pulmonary venous connection: one case report.

INTRODUCTION: The left partial anomalous pulmonary vein connection is a rare congenital heart disease, especially with intact atrial septum. Now we reported a case of the left superior pulmonary vein drainage to left innominate vein through a vertical vein, and corrected with video assisted thoracoscopy. CASE PRESENTATION: A-59-years old man diagnosed left anomalous partial pulmonary vein connection with presentation of short breathiness and palpation, and diagnosed with computer tomography pulmonary angiography. The operation was carried out under video assisted thoracoscopy with one manipulation incision and one observational incision, the vertical vein was dissected and anastomosis with left atrial appendage. The patients recovered smoothly and postoperative CTPA showed anastomosis ostium was unobstructed. CONCLUSION: The left lateral thoracotomy and video assisted thoracoscopic surgery is a feasible for correction of left PAPVC with intact interatrial septum without using CPB.

Hitherto Unreported Pattern of Complex Obstructive Partial Anomalous Pulmonary Venous Drainage with Dual Drainage of Accessory Pulmonary Veins.

Partial anomalous pulmonary venous drainage is a congenital cardiac disorder characterized by abnormal drainage of one or more pulmonary veins into the systemic circulation. It can be isolated or associated with other congenital cardiac anomalies, most commonly atrial septal defect and patent ductus arteriosus. The clinical presentation is variable and depends on the degree of shunting and associated cardiac anomalies. Many patients usually remain asymptomatic until late in life. In this article, we presented a complex case of obstructive partial anomalous pulmonary venous drainage with dual drainage of bilateral accessory pulmonary veins with intact interatrial septum in conjunction with a patent ductus arteriosus and a ventricular septal defect. This pattern is incredibly rare and to the best of our knowledge has not been previously reported. Computed tomography played a pivotal role in precisely elucidating the intricate anatomy in this case with a complex pattern of anomalous pulmonary venous drainage.

Modification of the Warden Procedure for Surgical Repair of Partial Anomalous Pulmonary Venous Connection.

Warden procedure is a popular technique for repairing partial anomalous pulmonary venous connection. We describe a modification of this technique for surgical repair of this condition by raising both a superior vena cava (SVC) flap and right atrial appendage flap to create a tension-free SVC-RA continuity (neo-SVC). The anomalous pulmonary veins are routed via the remanent of proximal SVC and baffled to the left atrium across a surgically created or enlarged atrial septal defect using autologous pericardium.

Hypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of "scimitar lung".

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiovascular condition in which some of the pulmonary veins drain into the systemic circulation. We report on the cadaveric dissection of a 71-year-old Caucasian male donor who died of chronic obstructive pulmonary disease with hypertension. We noted a faint incisional scar on the thorax extending from the parasternal region at the 4th intercostal level to the midaxillary line. Since the straight-line incision followed the ribs and the scar was quite faint, surgery probably occurred when the donor was young. We also observed numerous surgical interventions of the heart, lungs, and vasculature to correct various defects. The morphology of the heart was normal, but was shifted more to the right side. An atrial septal defect (ASD) was closed with sutures. The right superior pulmonary vein that drained into the superior vena cava (SVC) was ligated close to the SVC and the right inferior, left superior, and inferior pulmonary veins all drained directly into the left atrium. We noticed a dilated coronary sinus entering the right atrium adjacent to the ASD; the ostium of the coronary sinus noticeably lacked the normal valve-like structure. We initially thought the right lung was a "horseshoe" lung, but realised that it was a "hypogenetic" lung with PAPVR and an accessory diaphragm. Compared to the left, the right secondary bronchi were much narrower and branched uncharacteristically, as seen in hypogenetic lung syndrome. The inferior lobe was highly disorganised, severely hypoplastic, and exhibited uncharacteristic morphology. The superior bronchopulmonary segment was markedly hypoplastic. The posterior and medial basal segments were not only hypoplastic and slender, but also extended like a tail to the left pulmonary cavity behind the heart/pericardium and in front of the oesophagus and aorta. The right lung, though hypoplastic, demonstrated patent bronchi and the lobes were inflatable. Based on the hypogenetic lung and PAPVR, we conclude that the donor exhibited 'scimitar' lung.

A Rare Case of Partial Anomalous Pulmonary Venous Connection.

Partial anomalous pulmonary venous connection (PAPVC), in which the right and left lower pulmonary veins drain into the coronary sinus (CS), is very rare, and only one case has been reported previously. The diagnosis of PAPVC is difficult, as the symptoms may be not specific. Multidetector computed tomography (MDCT) angiography and MRI help in the diagnosis of congenital cardiac anomalies. Our case, who underwent closure of atrial septal defect (ASD) 31 years prior, presented with chest tightness and palpitation. MDCT angiography revealed PAPVC with both lower pulmonary veins draining into the CS. We performed surgical repair of PAPVC by the cut-back method. The postoperative course was uneventful.

Surgical repair of the adult form of scimitar syndrome.

Scimitar syndrome has various clinical presentations and anatomic features, and some cases are diagnosed in adulthood. Reconstruction using autologous tissue to reroute the right pulmonary venous blood flow to the left atrium is ideal. However, if the scimitar vein drains to the caudal segment of the inferior vena cava, reconstruction using prosthetic material may be necessary due to the distance between the left atrium and the scimitar vein. We describe the case of a 16-year-old boy with scimitar syndrome. We anastomosed the scimitar vein to the right atrium using an artificial graft and created an atrial septal defect for rerouting the right pulmonary venous blood to the left atrium. It has been 9 years since this procedure, and the patient has not experienced graft stenosis or thromboembolic events.

Complex complete transposition of great arteries and partial anomalous pulmonary venous connection with an intact interatrial septum-lessons learnt.

The association of pulmonary venous anomaly and a complex complete transposition of great arteries (d-TGA) is a rarity. Managing a combination of anomalies in a single setting is a surgical challenge. We report a very rare association of d-TGA with ventricular septal defect (VSD) and partial anomalous pulmonary venous connection (PAPVC) of the right pulmonary veins to the right atrium with an intact interatrial septum in a month-old infant. The child underwent a single-stage complete repair. Constant vigilance in the immediate postoperative period and early revision is mandatory for a good outcome.

Left upper lobe pulmonary adenocarcinoma with partial anomalous pulmonary venous connection in the same lobe: Case report / Bal felso lebenyi adenocarcinoma és pulmonális vénás fejlodési rendellenesség együttes elofordulása

Authors present a case of a 60-year-old male patient with left upper lobe cancer in association with partial anomalous pulmonary venous connection (PAPVC) in the same lobe. The hemiazygous vein joined the left superior pulmonary vein above the aorta in the thorax cavity draining into the left brachiocephalic vein causing left to right shunt flow. PAPVC was clearly identified intraoperatively and left upper lobectomy was performed as definitive solution for both. PAPVC was closed by stapler. To our knowledge 32 operated cases of lung cancer with PAPVC has been described in the literature (PubMed), including our patient.

An adult case with absence of left pulmonary artery and partial anomalous pulmonary venous connection: Case report and literature review.

Unilateral absence of pulmonary artery is a rare congenital disorder that can remain asymptomatic until adulthood. Absence of left pulmonary artery (ALPA) has been reported in one-third of these patients. We are the first to introduce an adult case of ALPA associated with partial anomalous pulmonary venous connection.

Sinus venosus ASD and PAPVC with constrictive pericarditis: a rare association.

This is a case of a young man who underwent preoperative computed tomography evaluation for sinus venosus type of atrial septal defect, and was found to have associated chronic constrictive pericarditis. Association of these two entities is rare and changes the management approach.

Partial Anomalous Pulmonary Venous Connection: A Great Imitator?

In isolated partial anomalous pulmonary venous connections (PAPVCs), an abnormal vein connects venous blood from the pulmonary circulation to the systemic circulation, resulting in an extracardiac shunt. A single aberrant pulmonary vein (PV) is usually hemodynamically insignificant, and affected patients are generally asymptomatic. We describe a young Caribbean-Black woman with an isolated, singular PAPVC from the left inferior PV to the left innominate (brachiocephalic) vein that was hemodynamically significant, obfuscated by recurrent pleural effusions from catamenial pleural endometriosis.

Outcomes of the Warden Procedure for Partial Anomalous Pulmonary Venous Drainage.

Partial anomalous pulmonary venous drainage (PAPVD) is a common congenital heart disease. If the insertion of an anomalous pulmonary vein (PV) is high into the superior vena cava (SVC), the traditional 1-patch or 2-patch surgical repair might be challenging. Unlike patch procedures, the cavoatrial anastomosis technique (Warden procedure) theoretically reduces complications such as sinus node dysfunction and venous obstruction. We investigate outcomes of the Warden procedure (WP) at a single institution. A total of 67 patients (42 males and 25 females) with PAPVD who underwent the WP between January 2011 and December 2018 were consecutively enrolled. The median age was 2.8 years (3-61 years old). 52 cases were younger than 18 years old. perioperative and follow-up clinical data were collected. In addition, we selected possible risk factors (a total of 18 risk factors) of SVC stenosis or obstruction. Univariate and multivariate analyses were conducted to confirm the independent risk factors. The average Cardiopulmonary bypass (CPB) time was 132.3 ± 46.3 min, and the aortic cross-clamp time was 85.2 ± 35.7 min. One patient died postoperatively in hospital. The mean follow-up time of the remaining patients (n = 66) was 15.8 ± 14.5 months (3-64 months). No abnormal cardiac function or sinus node dysfunction cases were identified during the follow-up. Of the survival patients, no venous obstruction was presented before the discharge. Numbers of patients identified with mild PV and SVC stenosis were 1 (1.5%) and 7 (10.6%, 4 of them turned negative eventually) at discharge. At the last follow-up, no PV obstruction was identified, whereas, 4 cases (6%) had SVC obstruction. Numbers of PV and SVC stenosis cases were 3 (4.5%) and 8 (12%). Univariate and multivariate analyses showed that only the diameter of SVC less than 10 mm was an independent risk factor for SVC stenosis or obstruction at the last follow-up. Warden procedure can effectively treat PAPVD (anomalous drainages into SVC), with satisfactory early and mid-term postoperative results. Different modifications of the Warden procedure may be selected according to the anatomic characteristics. The diameter of SVC less than 10 mm predicts SVC stenosis or obstruction after Warden procedure.

Azygos Vein to the Rescue of Warden Anastomosis in High Pulmonary Venous Drainage.

Partial anomalous pulmonary venous return is a congenital anomaly in which some of the pulmonary veins drain into the right atrium or one of its venous tributaries. Although excellent long-term results have been described for the classic two-patch technique repair in older patients, The Warden procedure has an important role in smaller and younger patients and those patients in whom the superior vena cava (SVC) may be small and the right-sided pulmonary veins may connect high, at or above the azygos vein. In addition to prevention of early and late sinus node dysfunction, the Warden procedure helps to avoid systemic or pulmonary venous obstruction if care is taken to ensure a tension-free Warden anastomosis. Patch extension or interposition of prosthetic grafts with no growth potential have been used in instances of high partial anomalous pulmonary venous connection (PAPVC) to SVC. We report three cases of high PAPVC to SVC using an innovative technique of incorporating the azygos vein into the Warden anastomosis for a tension-free suture line.

Repair of Sinus Venosus Defects With Partial Anomalous Pulmonary Venous Connection in Children by Modified Right Vertical Infra Axillary Thoracotomy.

OBJECTIVE: To evaluate the safety and reproducibility of modified right vertical infra axillary thoracotomy (RVIAT) for repair of sinus venosus defects with right-sided partial anomalous pulmonary venous connection (PAPVC) in children. METHODS: Between March 2017 and February 2018, we performed intracardiac repair for sinus venosus defects with right-sided PAPVC in 14 children through modified RVIAT. Median age and weight were 9.5 years and 21 kg, respectively. We modified RVIAT by avoiding central venous cannulation and used total peripheral venous cannulation (right internal jugular vein and right femoral vein). In all children double-patch technique was followed, using untreated autologous pericardium. RESULTS: Intracardiac repair was safely performed in all children. There was no conversion to another approach and there were no complications related to peripheral venous cannulation. All children were in sinus rhythm with no residual defects, with non-obstructive pulmonary venous drainage at the time of discharge and during subsequent follow-ups. CONCLUSIONS: Modified RVIAT can be safely performed for repair of sinus venosus defects with right-sided PAPVC, without compromising on the quality of repair. With this modification not only the intracardiac repair was easier, also it provided more working space with minimal rib spreading.

Left sided PAPVC with intact IAS-Surgically managed with vertical vein anastomosis to LA appendage: A rare case report.

INTRODUCTION: Partial anomalous pulmonary venous connection (PAPVC) is a rare entity. Only 10% of these are left sided. An intact atrial septum is further uncommon. PRESENTATION OF CASE: We present a case of left sided PAPVC with no atrial septal defect (ASD), who presented with effort intolerance and an unremarkable physical examination. Computed tomography pulmonary angiography (CTPA) confirmed the primary diagnosis as suggested by an initial 2-D echocardiography, and aided in better understanding of the anatomy. CONCLUSION: Patient underwent successful surgery through a simple & reproducible technique of anastomosis of vertical vein to left atrial appendage. Patient recovered uneventfully and was discharged on day 10.

A Partial Anomalous Pulmonary Venous Connection in a Severely Symptomatic Patient, Is Surgery Always Recommended?

Partial anomalous pulmonary venous connection (PAPVC) is a rare cardiac anomaly occurring when a pulmonary vein drains into the right atrium, coronary sinus or a systemic vein creating a left-to-right shunt. Symptoms develop from right-sided fluid overload and pulmonary vascular disease. We report a rare case of a severely symptomatic patient with an incidentally discovered PAPVC in the setting of underlying severe pulmonary hypertension from multifactorial severe restrictive lung disease. Despite his worsening symptoms, a multi-disciplinary meeting decided against surgical intervention. Nine months after the decision was made, the patient showed no signs or symptoms of clinical deterioration. Prior studies recommend surgery for PAPVCs with evidence of right ventricular dilation, mild-to-moderate tricuspid regurgitation, or early stages of pulmonary vascular disease. However, our case demonstrates how decision making should consider the shunt's contribution to the overall clinical picture and underlying comorbidities. If a decision is made to defer surgical intervention, strict follow up and repeat re-evaluations for possible risk re-stratification and surgery reconsideration are warranted.

Vena cava anomalies in thoracic surgery.

BACKGROUND: Vena cava anomalies are a rare group of anatomical variations due to an incorrect development of the superior or inferior vena cava during fetal life. They generally show no clinical relevance and the diagnosis is done due to the association with congenital heart diseases in most of cases. However, preoperative identification of these anomalies is mandatory for surgeons to proper surgical planning. If not recognized, lethal complications may occur, as already reported in literature. CASE PRESENTATION: We report a case series of three different unidentified vena cava anomalies in patients undergoing lung resection. These unrecognized anomalies led to minor complications in two cases and required an accurate intraoperative evaluation in another. A careful retrospective evaluation of preoperative radiological images showed the anomalies. CONCLUSIONS: A careful evaluation of the vena cava anatomy at pre-operative imaging is mandatory for thoracic surgeons to properly plan the surgery and avoid complications.