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In view of the existing controversy concerning the best perfusion technique during deep hypothermic circulatory arrest (DHCA) for neonatal heart operations, we examined intraoperative rSO2 to help define an optimal interval for an intermittent antegrade cerebral perfusion (IACP) strategy. Records of patients undergoing stage 1 palliation (S1P) and repair of total anomalous pulmonary venous return (rTAPVR) from 1996 to 2004 were reviewed. A total of 16 patients were identified (11 S1P, 5 rTAPVR) with complete data and long periods of DHCA. A decline in rSO2 of either 20% or below a value of 50 was considered significant. The rSO2 for all patients was evaluated after 5, 10, 15, and 20 minutes of DHCA for significant cerebral desaturation. The average rSO2 at the start of DHCA ranged from 45 to 89 for S1P and 35-86 for rTAPVR. Significant cerebral desaturation was observed in 25%-31% of patients after 5 minutes; 42%-44% of patients after 10 minutes; 58%-69% after 15 minutes; and 75%-83% after 20 minutes. Each neonate has a unique baseline cerebral saturation. Also, the response to DHCA varies among subjects as the rate of decrease of rSO2 was not uniform. Universally applying the same interval after which to perfuse the brain permits significant cerebral desaturation in a large percentage of patients. Cerebral oximetry may provide a guide for developing an individualized cerebral perfusion strategy.

Less than 7% patients with TAPVR survive into adulthood. Predictors of survive in untreated patients are: absence of concomitant CHD & atrial isomerism; large ASD; low pulmonary vascular resistance; mild/moderate TV incompetence. Experience of TAPVR repair in adults restricted by prevalence of case reports. Majority of repaired were ≥50 years old. There have been four case reports of untreated TAPVC diagnosed after 60 years of age, and three of them underwent successful surgery on 61[J Melki,1992], 63 [MH McMullan,1992] and 66 years of age[U Wetzel,2010]. Large surgical series presented by X Jian, 2012 [12 pts], S Talwar, 2008[27 pts], B Korbmacher, 2001[12 pts] and J Rodriguez-Collado, 1992 [19 pts]. Among case reports of TAPVR in adults, up to our knowledge, there are only 4 patients with TAPVR and with PA valve stenosis /RVOT obstruction.

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.

A 63-year-old female who had palpitations and dizziness was referred to our hospital because of cardiac enlargement shown on chest X-ray in a health examination. On chest X-ray, enlargement of protrusion of the right first and left first aortic arches was observed. Electrocardiogram revealed ectopic low part atrial rhythm, right …

We report the case of a young girl with a mixed total anomalous pulmonary venous return (cardiac and supracardiac) treated sequentially by partial neonatal surgery, and then catheterization at age 19 with installation of an Amplatzer device as a treatment of the remaining anomaly. We describe the usefulness of magnetic resonance imaging in both the diagnosis and follow-up of this anomaly.

An 8-day old male infant is sent to the emergency department by his pediatrician for weight loss, pallor, and respiratory distress. On arrival, the infant was found to be in shock. The infant was resuscitated and an echocardiogram revealed infradiaphragmatic total anomalous pulmonary venous return. This article will provide an overview of the prevalence, classification, clinical manifestations, diagnostic workup, and management of total anomalous pulmonary venous return.

A 2-hour-old term female infant presents with respiratory distress and oxygen desaturation requiring 100% oxygen. ### Prenatal history ### Birth history ### Presentation

Objective To evaluate whether pulse oximetry can be used as a screening test to detect congenital heart disease (CHD) in otherwise well newborns before nursery discharge. Methods Births at Lakeland Regional Medical Center (LRMC) who met inclusion criteria, had a single pulse oximeter reading before discharge. A normal reading was considered to be >94%. Abnormal readings were repeated on a different extremity and if still abnormal, an echocardiography was performed. The number of children with CHD and the number of echocardiograms were compared with the previous year at LRMC. Results There were 2114 infants enrolled, 88 echocardiograms (3.8%) were performed, and 43 were abnormal (yield = 48.9%), of which 12 required management (13.6%). The control group consisted of 2851 births, producing 108 echocardiograms (3.8%), 42 of which were abnormal (38.9%), and 13 required management (12.0%). No comparisons were significant. During both years, 3 children with cyanotic CHD were born. Routine pulse oximetry failed to detect a child with total anomalous pulmonary venous return. Not a single normal echocardiogram was generated by the study. Conclusion Routine pulse oximetry was nearly 100% specific for detecting cyanotic CHD; as a result there was no increase in the number of echocardiograms. Routine pulse oximetry did detect a child who might not have been detected otherwise but failed to detect one that should have been detected. (J Pediatr 2003;142:268-72)

Transesophageal echocardiography (TEE) during cardiac surgery is a routine procedure. The use of pediatric TEE probes is limited in small infants weighing less than 5 kg. Recent reports have shown the safety of monoplane intravascular ultrasound catheters in transesophageal echocardiograms. This report describes the case of a newborn with total anomalous pulmonary venous return who underwent cardiac surgery. A pre- and postbypass TEE examination was performed, with successful visualization of the cardiac anatomy and function and no complications.

We report a case of a neonate who presented with obstructed mixed total anomalous pulmonary venous return (TAPVR) and Williams syndrome. After initial repair of TAPVR, his aortic wall continued to thicken, resulting in aortic arch hypoplasia and near atresia of the entire course of the left main coronary artery requiring a novel type of repair at 5 months of age. Cardiovascular abnormalities are frequently associated with Williams syndrome. This is a report of a rare patient with both Williams syndrome and obstructed TAPVR.

A newborn with right isomerism, infracardiac total anomalous pulmonary venous return (TAPVR), and obstruction of the ductus venosus underwent stent implantation in the ductus venosus at 4 h after birth. This procedure averted early pulmonary venous obstruction. Since gradual neointimal proliferation subsequently occurred within the stent, the patient underwent stent balloon dilatation at 35 days of age and underwent surgical repair of the TAPVR with a Blalock-Taussig shunt operation at 53 days of age. Finally, the patient was discharged without any complications. Transthoracic echocardiography is very useful to confirm subsequent minimal change within the stent.

A newborn baby presented with respiratory distress, cyanosis and shock within 2 hours of birth. The cardiac evaluation showed supracardiac total anomalous pulmonary venous return with critically obstructed vertical vein. The baby underwent successful stenting of the vertical vein at 12 hours of life.

Congenital heart disease (CHD) occurs in 8 per 1000 live births, with approximately one third of these neonates requiring intervention in the first month of life. Neonates with respiratory distress, cyanosis, feeding difficulties, low cardiac output, or dysmorphic syndromes commonly have CHD. Clinical suspicion increases in a symptomatic infant with a heart murmur, but the presence or absence of a murmur does not assure either the presence or absence of significant congenital heart disease. Infants suspected to have CHD may be divided into premature and term infants, as well as infants with duct-dependent pulmonary blood flow, infants with duct-dependent systemic blood flow, and infants with unrestricted pulmonary blood flow. This article will also address the specialized clinical situations of total anomalous pulmonary venous return, transposition of the great arteries, and hypoplastic left heart syndrome with intact atrial septum.

Presented, is the case of a newborn with left-sided lung agenesis associated with total anomalous pulmonary venous return, atrial septal defect, polysplenia and hypospadias was described. Throughout the entire hospitalization period the newborn was in poor general condition, and dependent on mechanical ventilation with high oxygen concentrations. On day 20, the newborn died. According to the available literature, this case is unique in regard to the type and number of malformations.

We present an adult woman with total anomalous pulmonary venous return (TAPVR) and Mayer-Rokitansky-Kuster-Hauser syndrome that was diagnosed intraoperatively during a planned atrial secundum defect closure. Surgical repair of TAPVR was performed with good outcome.

症例は38歳, 男性である. TAPVR (Darling Ia 型) による急性心不全で緊急入院した. 肺静脈レベル (垂直静脈は短頸, 肺静脈閉塞なし), 心房レベル (ASDは28×24mm) に還流障害はなく肺血流増多であった. 根治手術は上方経路で行い, 術後3カ月目の心臓カテーテル検査で吻合部狭窄の所見はなかった. 心臓の脱転や両心房切開を行わない上方経路は視野も良く, 自然な位置での左房・総肺静脈吻合が可能である. また, 長期生存の最大の要因として肺血管抵抗の上昇がなかったことが考えられた.

Most patients with total abnormal venous connection (TAPVC) have no symptoms at birth, yet the majority die within the first year of life if surgical repair is not implemented. TAPVC is usually suspected in the neonatal period, and without surgical repair prolonged survival is quite exceptional. This kind of abnormality can be a real challenge for a non-pediatrics cardiologist today and can be easily misdiagnosed as a large secundum atrial septal defect if there is no sufficient suspicion of TAPVC. Therefore, a case of a 40-year-old female patient referred to our clinic with increasing shortness of breath is of great interest, as this patient was diagnosed with TAPVC.

A newborn girl with progressive respiratory distress and cyanosis was found to have severely obstructed supracardiac total pulmonary venous return (TAPVR). Stenting of the stenosis provided immediate and effective relief of the obstruction until corrective surgery was performed. Catheter intervention should be considered in high-risk infants with obstructed supracardiac TAPVR as part of the strategy for preoperative cardiovascular stabilization.

An alternative technique for the repair of supracardiac total anomalous pulmonary venous return (TAPVR) is described. The pulmonary venous confluence (CPV) is identified below the right pulmonary artery between the aorta and superior vena cava. The atrial incisions are similar to a septosuperior approach of the mitral valve, modifying the incision on the roof of the left atrium to be parallel to the incision in the CPV. The common pulmonary vein is anastomosed to the roof of the left atrium. This approach offers optimal exposure for the repair without distorting the cardiac structures.

Although indications for a Fontan procedure have broadened, some patients, in the past, were ineligible for the Fontan completion after a Glenn procedure and thus suffered the limitations of the Glenn procedure—namely desaturation, arrhythmia and reduced quality of life. If examined more closely, however, completion may yet be feasible for such patients. We present here a complex case of asplenia, dextrocardia and total anomalous pulmonary venous return (1b) where the Fontan procedure was successfully completed 12 years after the Glenn procedure. A unique surgical strategy incorporating intra-atrial total cavopulmonary connection and atrioventricular valve repair contributed to our success.

Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect in which the pulmonary veins fail to connect to the left atrium returning instead to the right atrium or one of its venous tributaries. Using linkage analysis in a large family with autosomal dominant transmission of TAPVR, we mapped a TAPVR locus on chromosome 4pl3-q12. Linkage analysis in additional families and analysis of two overlapping founder-effect haplotypes limit the TAPVR-1 interval to proximal and distal intervals, each of about 1 cM. Further study of TAPVR has focused on physical mapping of the region, Fluorescence in situ hybridization (FISH) to search for chromosomal abnormalities, and screening of candidate genes. As part of the search for candidate genes in the distal TAPVR-1 interval, I cloned and characterized a novel bicoid-related homeodomain-encoding gene named Sakasama (SAMA-1). Expression of SAMA-1 is tissue specific and developmentally regulated, with highest expression in the brain and lungs. Use of alternative transcription initiation sites, which may encode long and short forms of the protein, is also tissue specific and developmentally regulated. No mutations in this gene were discovered in any TAPVR patients. I have expressed this gene in Xenopus to test the hypothesis that it functions as an inhibitor of other bicoid-related homeodomain genes. Other work toward my thesis focused on an X-linked recessive, neonatal lethal cardiomyopathy. called isolated noncompaction of the ventricular myocardium (INVM). We mapped this disorder to Xq28 using linkage analysis in a family with six affected males, four of whom died in the first year. The gene mapped near the Barth syndrome locus, which shares some phenotypic overlap, including cardiomyopathy. A glycine-to-arginine mutation was discovered in a conserved domain of the same protein defective in Barth syndrome, called Tafazzin. The INVM phenotype is more severe than most cases of the allelic Barth syndrome, which may be explained by the genotypes seen in each disorder. Efforts to elucidate the cellular function of Tafazzin by disruption of the yeast homologue, YPR140W have been initiated. Recent reports suggest the protein may function as an acyltransferase.

Total anomalous pulmonary venous return (TAPVR) is a rare form of cyanotic congenital heart disease which, without surgical correction, has a high mortality rate in the first year of life. It usually occurs without a family history, and is generally thought to have a low recurrence risk. However, 15 instances of familial TAPVR have been reported previously, suggesting a genetic cause in some cases. We report on a large family in which TAPVR appears to be determined multifactorially or as an autosomal dominant trait with variable expressivity and reduced penetrance.

Familial recurrence of congenital heart disease (CHD), in particular, d-transposition of great arteries (d-TGA) is rare. However, there have been several reports in the literature of sibling recurrence of total anomalous pulmonary venous return (TAPVR). This is the first case report in the literature, describing mother to offspring recurrence of d-TGA. We describe two cases of non-syndromic CHD with mother to offspring and sibling recurrence. The first case is an antenatally diagnosed d-TGA on fetal echocardiogram at 25 weeks of gestational age in the offspring of a 30-year-old mother with d-TGA. The second case is a sibling reoccurrence of TAPVR diagnosed antenatally at 30 weeks of gestational age, with supradiaphragmatic TAPVR on fetal echocardiogram in a mother, whose first child was diagnosed with infradiaphragmatic TAPVR in infancy.

A 37-year-old man presented at the emergency department with recent-onset dyspnea (New York Heart Association functional class IV) and abdominal distention. He had no prior history of exercise intolerance, shortness of breath, cyanosis, or palpitation. In the emergency department, tachycardia and engorged jugular veins were observed, and breath sounds were decreased on the left lower lung field, with no crackles. There was no cardiac murmur. The liver was palpable in the distended abdomen. Chest radiography demonstrated marked cardiomegaly with a snowman appearance of increased pulmonary vascular markings (Figure 1, left). The …

TAPVR typically presents in early infancy due to excessive pulmonary blood flow and mild cyanosis. Presentation is earlier when there is obstruction to pulmonary venous flow where neonates present with severe cyanosis and cardiogenic shock, surgical repair must be planned immediately. Mixing of well oxygenated blood through the anomalous connection of pulmonary veins with the systemic blood in the right atrium results in similar oxygen saturation in all cardiac chambers and arteries, therefore pre and post ductal oxygen saturations are equal. Increased blood flow across the right heart causes delay in closure of the pulmonary valve, presenting as fixed splitting of second heart sound as well as systolic flow murmur over the pulmonic region. Supra-cardiac drainage of anomalous pulmonary veins to the superior vena cava result in figure-eight or snow man appearance of the cardiac silhouette on CXR.

Total anomalous pulmonary venous return (TAPVR) is an uncommon cause of cyanotic congenital heart disease in the neonatal period. This diagnosis is often made by echocardiography. We present two cases where echocardiography was not confirmatory. Computerized tomography (CT) scan of the chest and abdomen with contrast was performed instead of cardiac catheterization to establish the diagnosis. We suggest that CT with contrast is a noninvasive modality to obtain anatomic details of pulmonary venous drainage in TAPVR when echocardiography is inconclusive.

Among 57 neonates undergoing repair of total anomalous pulmonary venous return with severe pulmonary venous obstruction from 1980 through 1989, date of operation (1980 to 1984), preoperative hemodynamic instability, and failure to monitor pulmonary artery pressure postoperatively were risk factors for death. Thus, among the 30 patients having repair between 1985 and 1989, the 55-month survival rate including hospital deaths was 83%.

Horseshoe lung is a rare pulmonary anomaly characterized by fusion of the posterobasal portions of the right and left lung behind the heart anterior to the aorta and the spinal column. This anomaly is almost always associated with lung hypoplasia. The case of a newborn who had horseshoe lung with left lung hypoplasia and cardiac-type total anomalous pulmonary venous return is reported.

Cardiovascular imaging is a rapidly evolving field that requires familiarity with the appearances of pediatric and adult cardiovascular diseases on chest radiographs as well as images obtained with computed tomography, magnetic resonance imaging, and angiography. To accurately identify congenital abnormalities affecting the heart and vessels of the thorax, radiologists must recognize the imaging features and understand their pathophysiologic origin. The cardiovascular imaging signs of congenital anomalies that are most often seen in radiologic practice include the egg on a string (seen in transposition of the great arteries), snowman (total anomalous pulmonary venous return), scimitar (partial anomalous pulmonary venous return), gooseneck (endocardial cushion defect), figure of three and reverse figure of three (aortic coarctation), boot-shaped heart (tetralogy of Fallot), and box-shaped heart (Ebstein anomaly). © RSNA, 2007

Intracardiac repair for complex congenital heart defects in premature neonates with very low birth weight (VLBW) is still a challenge to pediatric cardiac surgeons. We report the successful rerouting of cardiac type total anomalous pulmonary venous return (TAPVR) in a premature newborn (36 th gestational week) with VLBW (1250 g). She had severe hypoxemia and low cardiac output despite medical treatment. Rerouting surgery of TAPVR was performed under deep hypothermia circulatory arrest at the age of 20 days. The sternum was left open and approximated 2 days later. Follow-up echocardiography showed good ventricular function without pulmonary venous obstruction. The endotracheal tube was removed 7 days postoperatively. She was then discharged without complication. In conclusion, with improved cardiopulmonary bypass technique and perioperative care, open heart surgery can be performed in premature newborns with VLBW. [ J Formos Med Assoc 2007;106(2 Suppl):S23-S26]