Two-Stage Intracardiac Repair for Total Anomalous Pulmonary Venous Return (Ib+Ib) with a Persistent Left Superior vena cava and an Enlarged Coronary Sinus / 日本心臓血管外科学会雑誌

This study reports the case of a 0-day-old girl who was transferred to our hospital for suspected total anomalous pulmonary venous return due to her postnatal cyanosis. After she was presented at our hospital, pulmonary vein stenosis was diagnosed and emergency surgery was planned. Preoperative computed tomography showed abnormal perfusion of the pulmonary veins into the left and right superior vena cava separately on the left and right sides. Given that the persistent left superior vena cava was refluxing into the coronary sinus, the coronary sinus was enlarged greatly, and the left atrial volume was small. In the neonatal period, the right pulmonary vein was anastomosed to the right atrium, and the left pulmonary vein was anastomosed to the unroofed coronary sinus. Thereafter, at 4 months of age, the patient underwent two-stage intracardiac repair with re-routing of the right pulmonary vein and extracardiac ligation of the left vena cava. The postoperative course was good. Intracardiac repair via a two-stage surgery was effective for total anomalous pulmonary venous return (Ib+Ib) with a persistent left superior vena cava and an enlarged coronary sinus.

A Case of Partial Anomalous Pulmonary Venous Return in an Adult Patient / 日本心臓血管外科学会雑誌

Most cases of partial anomalous pulmonary venous return are associated with atrial septal defect. We however report a surgical case of partial anomalous pulmonary venous return that was diagnosed in an adult without atrial septal defect. The patient was a 44-year-old man who presented with hemoptysis. Computed tomography revealed an absent left pulmonary artery. We performed bronchial artery embolization. Partial anomalous pulmonary venous return (Qp/Qs 3.33) in which the right pulmonary vein returned to the superior vena cava and moderate aortic regurgitation were diagnosed by computed tomography and transthoracic echocardiography ; this was an indication for surgery. Approximately 2 months after the emergency hospitalization due to hemoptysis, we performed a modified Warden procedure and aortic valve replacement. Postoperative computed tomography showed good reconstruction of the superior vena cava and right upper pulmonary vein. He was discharged on postoperative day 38.

Minimally Invasive Cardiac Surgery for Partial Anomalous Pulmonary Venous Return to the High Portion of the Superior Vena Cava / 日本心臓血管外科学会雑誌

We report a case of minimally invasive cardiac surgery (MICS) for partial anomalous pulmonary venous return (PAPVR) to the high portion of the superior vena cava. A 34-year-old female was referred to our hospital for exertional chest oppression, and was diagnosed with PAPVR and a sinus venosus atrial septal defect. Two pulmonary veins were connected to the superior vena cava (SVC) : one to the SVC-atrial junction and the other to the high SVC adjacent to the azygos connection. We performed an intracardiac repair through a small right axillary incision. The postoperative course was uneventful. MICS may become a useful option for PAPVR repair.

Outcomes of Surgery for Total Anomalous Pulmonary Venous Return without Total Circulatory Arrest

BACKGROUND: Recent developments in surgical techniques and hospital care have led to improved outcomes following total anomalous pulmonary venous return (TAPVR) repair. However, the surgical repair of TAPVR remains associated with a high risk of mortality and need for reoperation. We conducted this retrospective study to evaluate mid-term outcomes following in situ TAPVR repair without total circulatory arrest (TCA), and to identify the risk factors associated with surgical outcomes. METHODS: We retrospectively reviewed 29 cases of surgical intervention for TAPVR conducted between April 2000 and July 2015. All patients were newborns or infants who underwent in situ TAPVR repair without TCA. RESULTS: Four anatomic subtypes of TAPVR were included in this study: supracardiac (20 cases, 69.0%), cardiac (4 cases, 13.8%), infracardiac (4 cases, 13.8%), and mixed (1 case, 3.4%). The median follow-up period for all patients was 42.9 months. Two (6.9%) early mortalities occurred, as well as 2 (6.9%) cases of postoperative pulmonary venous obstruction (PVO). Preoperative ventilator care (p=0.027) and preoperative PVO (p=0.002) were found to be independent risk factors for mortality. CONCLUSION: In situ repair of TAPVR without TCA was associated with encouraging mid-term outcomes. Preoperative ventilator care and preoperative PVO were found to be independent risk factors for mortality associated with TAPVR repair.

Multiple Detector Computed Tomography Evaluation of Congenital Pulmonary Venolobar Syndrome: Scimitar Syndrome with Pulmonary Arterio-Venous Fistula.

The term dysmorphic lung has been introduced to describe any complex congenital malformation that involves both abnormal pulmonary vascular morphology and disordered growth of the entire lung. The major group within this definition of the dysmorphic lung is the scimitar syndrome. Scimitar syndrome is a rare anomaly consisting of partial anomalous pulmonary venous return into the supra or infradiaphragmatic portion of the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply from the aorta or one of its branches to the right lung. It can be associated with congenital cardiovascular defects namely dextrocardia, coarctation of the aorta, Tetralogy of Fallot, patent ductus arteriosus, atrial or ventricular septal defect, and right pulmonary artery hypoplasia. Associated pulmonary anomalies are lung hypoplasia, sequestration, and tracheobronchial anomalies. Thus, it can manifest with heart failure and recurrent pneumonia. Other reported associated anomalies include bronchogenic cysts, horseshoe lung, accessory diaphragm, and hernias. We report an 8-year-old boy with complaint of chest pain, who was diagnosed as having scimitar syndrome with rare association of pulmonary arteriovenous fistula within the involved lung and role of newer faster multiple detector computed tomography scanner in its evaluation.

Clinical profile of patients with Total Anomalous Pulmonary Venous Return and their short term outcome in pediatric cardiac centre at Dhaka Shishu Hospital.

A5Background: Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital cardiovascular anomaly with poor natural prognosis without proper intervention. It has been detected more frequently in recent year due to the advent of echocardiography. The aim of this study is to evaluate the clinical manifestations, age at diagnosis and short term outcomes in TAPVR patients. Methods: From 1st January 2013 to 31st December 2013, a total of 34 cases with TAPVR were admitted in pediatric cardiac centre at Dhaka Shishu Hospital, Dhaka, Bangladesh. All of them were evaluated with 2-dimensional (2-D) and color Doppler echocardiography examination. CXR and ECG were also done. Patient’s sex, age at diagnosis, types of TAPVR, clinical manifestations, radiological finding, ECG findings and outcomes were compiled and analyzed. Results: In 34 patients with TAPVR, 23 (67.6%) were male and 11 (32.4%) were female with male to female ratio of 2.09:1. Most of the patients were diagnosed between 0-6 months of age that is 13 (38.2%) cases were in 0-2 month’s age group, 14 (41.2%) cases were in more than 2 month’s to 6 month’s age group. Tachypnea and cyanosis were more common symptoms. The types of TAPVR was supra-cardiac 18 (52.9%), cardiac 11 (32.4%), infra-cardiac 3 (8.8%) and mixed in 2 (5.9%) cases. Pulmonary hypertension was present in 31 (91.2%) of 34 cases. Among them, 20 (58.8%) patients had severe pulmonary hypertension. The most common associated intra-cardiac lesions of TAPVR patients were ASD 13 (38.2%) and PFO 13 (38.2%). ECG findings of TAPVR, 18 (52.9%) patient had right axis deviation (RAD), right ventricular hypertrophy (RVH) and 14 (41.2%) had right axis deviation (RAD), right ventricular hypertrophy (RVH), right atrial enlargement (RAE). X-ray findings of TAPVR patients, 32 (94.1%) patients had Cardiomegaly and increased pulmonary vascularity. Among admitted patient, 3 (8.8%) patients died due to pneumonia and intractable heart failure, 31 (91.2%) patients referred to advanced cardiac centre for operative treatment. Conclusions: Tachypnea and Cyanosis were an obvious clinical symptom of TAPVR. 2-D and color Doppler echocardiography can provide quick and accurate diagnostic information of TAPVR. Death rate is high in TAPVR patient in spite of adequate medical management. So, early detection and definitive surgical treatment of TAPVR is much needed.

Síndrome de la cimitarra: serie de casos / Scimitar syndrome: a case series

Introducción: El síndrome de la cimitarra es una compleja anomalía congénita del desarrollo pulmonar, infrecuente y de múltiples variables. Se denomina así por su semejanza radiológica con la clásica espada torcida. Su característica definitoria es el drenaje pulmonar anómalo. Se asocia con diversas malformaciones cardiotorácicas y un amplio espectro de manifestaciones clínicas. Serie de casos: Se reporta una serie proveniente de la base de datos del Hospital Infantil de México Federico Gómez, revisión del periodo 2009-2013. Se encontraron nueve pacientes con diagnóstico de síndrome de la cimitarra. Se recabaron del expediente médico las características demográficas, estado clínico y parámetros hemodinámicos reportados. Conclusiones: En la presente serie llaman la atención ciertas diferencias entre el grupo de estudio y lo reportado en la literatura internacional. Se encontró que la mayoría de los pacientes fueron del sexo femenino, diagnosticados entre el primero y el vigésimo mes de vida. En el momento del estudio estaban asintomáticos; la mitad contaba con antecedentes de enfermedad respiratoria y el total con hipertensión pulmonar. La tercera parte del grupo requirió manejo quirúrgico.

Background: Scimitar syndrome is a rare and complex congenital anomaly of the lung with multiple variables and is named for its resemblance to the classical radiological crooked sword. Its defining feature is the anomalous pulmonary drainage. It is associated with various cardiothoracic malformations and a wide spectrum of clinical manifestations. Case series: Nine patients diagnosed with scimitar syndrome found in the database of Hospital Infantil de México between 2009 and 2013 were reviewed. Demographic records, clinical status and hemodynamic parameters reported were collected. Conclusions: This case series called attention to certain differences between our group of patients and those reported in the international literature. Patients were predominantly female and were diagnosed between 1 and 20 months of life. All were asymptomatic at the time of the study. Half of the patients had a history of respiratory disease and all patients had with pulmonary hypertension. Surgical management was required in on-third of the patient group.

Total Anomalous Pulmonary Venous Return in Siblings

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR have been reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery was performed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.

Signo de la cimitarra / Scimitar sign

The scimitar sign refers to the image resembling a short, curved Turkish sword called scimitar, created by an anomalous pulmonary vein. It can be seen in chest radiography, computed tomography and magnetic resonance. It forms part of the scimitar syndrome, characterized by partial or total anomalous pulmonary venous drainage, associated with hypoplasia of the right lung and dextrocardia. Depending on the magnitude of the right-to-left shunt, it may present in children with pulmonary hypertension and right cardiac failure, or as asymptomatic imaging findings in adults.

El signo de la cimitarra es la imagen creada por una vena pulmonar anómala similar a una espada turca, corta y curva llamada cimitarra. Se puede ver en radiografía de tórax, tomografía computada y resonancia magnética. Forma parte del síndrome de la cimitarra, caracterizado por un drenaje pulmonar anómalo, parcial o total, asociado a hipoplasia del pulmón derecho y dextrocardia. Dependiendo de la magnitud del shunt de derecha a izquierda producido, se puede presentar en edades infantiles con hipertensión pulmonar y falla cardiaca derecha, o en adultos, de manera asintomática, como hallazgo imagenológico.

Partial Anomalous Pulmonary Venous Return into Coronary Sinus with Intact Atrial Septum

We report a case of partial anomalous pulmonary venous return where the right upper and lower pulmonary veins drain into the coronary sinus with right-to-left shunt via patent foramen ovale. To our knowledge, this is the uncommon case where the interatrial septum is intact. The diagnosis was initially made by transthoracic echocardiography and transesophageal echocardiography with the infusion of agitated saline and confirmed by pulmonary artery angiography. Curative operation could not be performed because of the irreversible pulmonary hypertension.

Long-term Clinical Study of Total Anomalous Pulmonary Venous Return

PURPOSE: Total anomalous pulmonary venous return is a rare congenital heart disease, which is fatal if untreated, especially, if the obstruction in pulmonary venous return is obstructed. With the technical development of echocardiogram, we are now able to do an early diagnosis if TAPVR and perform surgery. Accordingly, this report was aimed to evaluate patients with TAPVR as well as evaluate the problematic factors in order to contribute to future treatment. METHODS: From Sept. 1st, 1985 to June 30, 1999, we retrospectively evaluated 107 patients who were diagnosed TAPVR by echocardiogram and catheterization and angiogram. TAPVR in complex heart diseases were excluded. RESULTS: The patients were composed of 64 boys and 43 girls with the mean age of 206 days. The subtype of TAPVR is as follows : supracardiac type 45%, cardiac type 33%, infracardiac type 9% and mixed type was 13%. In 39 patients(36%), pulmonary venous obstruction was detected by echocardiogram and all the patients with infracardiac type TAPVR, except one patient, revealed obstructive pulmonary venous return. Surgery was performed in 96 patients and the mean age at operation was 270 days old. The operative mortality was not different statistically among subtypes(P=0.212) and also was not different between obstructive type and nonobstructive type(P=0.370). The mean age at operation from 1994 to 1999 was younger than that of up to 1994. Pulmonary vein stenoses after operation were detected in 14 patients(14.6%) and only in 3 patients developed, pulmonary vein stenoses at the same site of previous ones. Thus, pulmonary vein stenoses after operation could be mainly considered as secondary due to operation. CONCLUSION: The results from our study reveal that operative results for TAPVR were much improved with early diagnosis and operation and those results were not dependent on the subtypes of TAPVR. Postoperative pulmonary vein stenosis was a poor prognostic factor and for early detection, catheterization and angiogram could be recommended if there is suspicion.

A Case of Three-Dimensional Image Reconstruction of Pulmonary Venous Return

A case of supracardiac type of total anomalous pulmonary venous return into the superior vena cava is reported which was successfully diagnosed using spiral CT and three-dimensional image reconstruction. Spiral CT with three-dimensional reconstruction is non-invasive method of visualizing the vascular system and in some instances can replace conventional angiography in pediatric patients.

A Case of Surgically Corrected-Combined form of Total Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return(connection) is a congenital heart disease. Especially, the reports of the patients with double connections of all pulmonary veins were rare. An infant who underwent operation for total anomalous pulmonary venous return had double drainge of all pulmonary veins to both left vertical vein and anomalous descending vein. This second lesion was not diagnosed preoperatively, but in operative field. Operative treatments were performed by ligation of left vertical vein and central pulmonary vein-to-left atrium anastomosis without ligation of the descending anomalous vein. For these less common variants fo total anomalous pulmonary venous return,a more detailed and precise understanding of morphology can facilitate accurate surgical repair. And also, early detection of the disease is an another issue for a good prognosis.

Clinical Study on Total Anomalous Pulmonary Venous Return

Clinical study was performed on 42 patients diagnosed as TARVR at the Departmet of pediatrics, Seoul National University Hospital, from January, 1966 to June, 1982. The results are as follows, 1) Of the 25 cases with isolated TAPVR, age distribution was from 10 days to 10 years. Seventeen cases were male and eight cases were female. Of the 24 cases with complicated TAPVR, age distribution was from 2 days to 22 years. Eighteen cases were male and six cases were female. 2) The type of TAPVR was supracardiac in 29 cases (59.1%), cardiac in 10 cases (24.4%), infradiaphragmatic in 3 cases (6.1%) and mixed in 5 cases (10.2%). 3) Birth weights of patients were within normal limits except 1 case. 4) The most freuent complaints at diagnosis were tachypnea and cyanosis. 5) PDA was the most frequently associated intracariac anomaly (54.1%) which was followed by pulmonary stenosis (16.6%), single atrium (16.6%) and single ventricle (16.6%). 6) Of the 25 cases with isolated TAPVR, chest roentgenographic findings were cardiomegaly in 22 cases (88%) and increased pulmonary vascularity in 23 cases (92%). 7) Of the 25 cases with isolated TAPVR, the ECG showed right axis deviation in 22 cases (88%), right atrial enlargement in 13 cases(52%), right ventricular hypertrophy in 24 cases (96%) and combined ventricular hypertrophy in 1 cases (4%). 8) Of the 23 cases with isolated TAPVR, echocardiographic finding showed increased dimension of the right ventricle in all cases. Of the 39 cases with TAPVR, the common venous chamber was visualized posterior to the left atrium in 14 cases(35.8%). 9) Cardiac catheterization was performed in 24 cases with isolated TAPVR. Pulmonary hypertension was observed in 19 cases (79.1%). 10) Fourteen patients with isolated TAPVR were operated. Three patients died.