Common Atrium Research Articles

Dextrocardia with Cor Biloculare in a 44 Year Old Women

Cor biloculare, a two-chambered heart, is a rare congenital anomaly of the heart. It’s characterized by a single or common atrium communicating with a single or common ventricle by a single atrio-ventricular valve. Most patients with cor biloculare die within neonatal period or even in fetal period, and very few survivors could grow up to adult. In this article, we report an adult case with dextrocardia and cor biloculare, who survived to the 44 years of life.

Effect of mechanical assistance of the systemic ventricle in single ventricle circulation with cavopulmonary connection

Previous attempts to support single ventricle circulation mechanically have suggested that acustom-built assist device is needed to push, rather than pull, through the pulmonary circulation. We hypothesized that using a conventional ventricular assist device, with or without conversion of a total cavopulmonary connection to a bidirectional Glenn cavopulmonary connection, would allow assistance by pulling blood through the circuit and improve the cardiac index (CI). Cavopulmonary connections were established in each of 5 Yorkshire pigs (25 kg) using ePTFE conduits in a Y configuration with appropriate clamping of the limbs of the Y to achieve a total cavopulmonary Fontan connection (TCPC), superior vena cava cavopulmonary connection (SVC Glenn), and inferior vena cava cavopulmonary connection (IVC Glenn). A common atrium had been established previously by balloon septostomy. Mechanical circulatory assistance of the single systemic ventricle was achieved using a centrifugal pump with common atrial inflow and proximal ascending aortic outflow. The CI was calculated using an ultrasonic flow meter placed on the distal ascending aorta and compared between the assisted and nonassisted circulation for 3 conditions: TCPC, SVC Glenn, and IVC Glenn. The mean pulmonary artery pressure, common atrial pressure, arterial oxygen saturation, partial pressure of arterial oxygen, and oxygen delivery were calculated. The unassisted SVC Glenn CI tended to be greater than the TCPC or IVC Glenn CI. Significant augmentation of total CI was achieved with mechanical assistance for SVC Glenn (109% ± 24%, P=.04) and TCPC (130% ± 109%, P=.01). The assisted CI achieved at least a mean baseline biventricular CI for all 3 support modes. Oxygen delivery was greatest for assisted SVC Glenn (1786 ± 1307 mL/L/min) and lowest for TCPC (1146 ± 386 mL/L/min), with a trend toward lower common atrial and pulmonary artery pressures for SVC Glenn. SVC bidirectional Glenn circulation might allow optimal augmentation of the CI and oxygen delivery in a failing single ventricle using a conventional pediatric ventricular assist device. The results from our model also suggest that the Fontan circulation itself can be supported with systemic ventricular assistance of the single ventricle.

Ventricular Fibrillation Following Synchronized Cardioversion in Junctional Ectopic Tachycardia: A Pediatric Case Report

Junctional ectopic tachycardia (JET) has been observed in up to 11.4% of pediatric patients after cardiac surgery and is the most common tachyarrhythmia during the early postoperative period. 1 Following surgery, the heart is vulnerable to the negative effects of a high heart rate and atrioventriular (AV) asynchrony, which can lead to hemodynamic instability, decreasing cardiac output. Accurate identification of JET is critical because it is not amenable to synchronized cardioversion and is an important factor in postoperative morbidity. 1 The following case is an example of the cardioversion attempt of a perfusing tachyarrhythmia mistakenly identified as supraventricular tachycardia (SVT).A 5-month-old, 8.7-kg, female infant underwent surgical treatment of complete atrialventricular septal defect with a common atrium, left-looped ventricles, pulmonary atresia with confluent pulmonary arteries, and dextrocardia. A Glenn shunt and AV valvuloplasty was performed with a cardiopulmonary bypass time of 225 minutes and aortic cross-clamp time of 47 minutes.Adenosine was successfully used in the operating room for SVT after cardiopulmonary bypass was discontinued. Intermittent tachycardia continued throughout the first postoperative night. The morning of postoperative day 1, the patient was tachycardiac (>200/min) with a variable arterial blood pressure. Supraventricular tachycardia was suspected, and the child was cardioverted using 0.5 J/kg of body weight (Figure 1). Immediately afterward ventricular fibrillation occurred without measurable blood pressure or detectable pulse. Defibrillation using 2 J/kg of body weight was immediately performed with restoration of tachyarrhythmia (220/min) with variable blood pressure present (Figure 2). Cardiologists were contacted and consultation identified the rhythm as JET, not SVT. The patient was cooled to a temperature of 35.5°C via environmental exposure. Potassium and magnesium levels were normalized and the child was given amiodarone 5 mg/kg body weight over 1 hour, with eventual restoration of sinus rhythm.Junctional ectopic tachycardia is associated with AV asynchrony, which may lead to hypotension. The rhythm does not respond to electrical cardioversion. 2 The dysrhythmia has been commonly observed following cardiac surgery, and its presence may be a marker of an untoward effect of the surgical procedure. 3 Risk factors for the development of JET include young age (<30 days), weight less than 4 kg, aortic cross-clamp time more than 60 minutes, cardiopulmonary bypass time more than 100 minutes, and postoperative magnesium and potassium levels that are lower than normal levels.4, 5 Identification of JET is crucial for proper treatment. When AV dissociation is present, cannon A waves will be apparent in the waveforms of central venous pressure monitoring, such as right- and left-atrial lines, and P waves will not be apparent on continuous electrocardiographic monitoring.Treatment of JET primarily involves support and restoration of hemodynamics. If the rhythm is slow and blood pressure is acceptable, then not treating the arrhythmia may be entertained. In cases of hemodynamic instability, treatment of JET includes overdrive pacing, avoidance of hyperthermia, optimization of sedation, pacer control, and administration of amiodarone.2–7 Optimizing electrolytes and decreasing inotropic therapy may also be indicated.4–7Interdisciplinary teams caring for critically ill pediatric cardiac surgery patients in the postoperative period should be aware that JET is a common rhythm for patients undergoing repairs near the AV node. Supraventricular tachycardia also can occur during this same timeframe and had been successfully treated in the patient with chemical cardioversion (adenosine) in the immediate postbypass period. Although suspecting SVT as the tachyarrhythmia in the patient was reasonable, on reflection, seeking expert consultation before proceeding to cardioversion would have been preferable. The patient had a perfusing rhythm at the time that may have allowed time for a cardiology consult, assessment, and development of a treatment plan. A 12-lead electrocardiogram also could have aided the assessment.Differences between SVT and JET could have served as cues to which rhythm was present. P waves are absent in both rhythms, and the rates are often more than 200/min in children younger than 1 year. Supraventricular tachycardia, however, usually has a fixed rate that is not changed by stimulation, temperature, or pain medications. The JET rate, on the contrary, can vary up and down on the basis of the patient’s condition. Therefore, JET is treated with cooling, sedative agents, and interventions that decrease metabolic demands.6,7 Those who care for pediatric patients at risk of these arrhythmias can use this knowledge to help inform the collaborative decision-making process for interventions.Proper and timely identification of JET is necessary to avoid morbidity and mortality of postoperative cardiac pediatric patients. Anticipating potential changes in the patient’s condition is critical so that evidence-based interventions can be initiated. Interdisciplinary team members caring for this population of patients need expert assessment skills and collaborative processes in place when complexities arise to ensure excellent care for pediatric patients and positive patient outcomes.

Partial endocardial cushion defect combined with persistent left superior vena cava to left atrial roof & cor triatriatum in an adult patient

Methods and results We report here with a case corresponding to the above disease entity, in which pulmonary veins were drained into a separate posterior chamber, which was connected to the common atrium, thus forming cor triatriatum. A 42-yearold Asian female was consulted for operation of p-ECD and PLSVC, which were detected during the evaluation of complete heart block. She complained of worsening dyspnea and syncope attack for recent one year. The PLSVC had no connection with right sided SVC and was thought to be drained into the left atrial roof directly. The operation was carried out under standard hypothermic cardiopulmonary bypass. A large common atrium without septal remnant was encountered on opening right atrium. The coronary sinus opening was absent and PLSVC was directly drained into atrial roof, to the right of the left atrial appendage. An opening measuring 3x3cm, which at first was confused as atrial septal defect, was seen to the right of PLSVC. An accessory chamber, draining all four pulmonary veins was connected to the common atrium by this opening. After closing mitral valve cleft, a generous intraatrial patch made of bovine pericardium was used to partition the common atrium. The opening connecting pulmonary venous chamber to the atrium was widely extended inferiorly and the upper part of the opening was partly closed to minimize distortion of the patch. The left atrial appendage was also incorporated into the right side of patch to simplify the baffling procedure. The tricuspid annuoplasty was carried out and permanent epicardial pacemaker was implanted n the end. The patient recovered without events.

Surgical Correction of Common Atrium without Noncardiac Congenital Anomalies

Common atrium (CA) is a rare congenital heart defect. We reviewed our experience of surgical treatment of CA and summarize the clinical features of CA and the key techniques for surgical correction. Between August 1984 and August 2010, 37 consecutive cases of CA underwent corrective surgery. There were no clinical findings of Down, asplenia-polysplenia or Ellis-van Creveld syndromes in all cases. Mitral valvuloplasty was performed in 34 cases, and tricuspid valvuloplasty in 20 cases. Complete closure of a mitral cleft was required in 32 cases. All new atrial septa were reconstructed using patches. Follow-up period ranged from 1 to 20 years. There were no hospital deaths or conduction system block. After surgery, mild mitral insufficiency was observed in only one case. Two cases had moderate-severe mitral insufficiency at postoperative years 1 and 3, respectively, that required mitral valve replacements. One patient died of low cardiac output syndrome after reoperation. Long-term survival after surgical correction of CA is good. Routine closure of a cleft in the mitral valve is very important for successful surgery.

Decompression of Thoracic Duct: New Approach for the Treatment of Failing Fontan

In failing Fontan circulation, the elevated central venous pressure increases lymphatic production and simultaneously retards lymphatic return to the central venous system, thus unbalancing lymphatic homoeostasis. To improve lymphatic drainage, a new concept based on decompression of the thoracic duct to the lower pressure levels of the common atrium, with concomitant increase of preload, has been developed. The thoracic duct, which in the majority of patients enters the circulation at the left subclavia-jugular junction, is decompressed by diverting the innominate vein directly to the common atrium.

On the role of intrinsic and extrinsic forces in early cardiac S‐looping

Looping is a crucial phase during heart development when the initially straight heart tube is transformed into a shape that more closely resembles the mature heart. Although the genetic and biochemical pathways of cardiac looping have been well studied, the biophysical mechanisms that actually effect the looping process remain poorly understood. Using a combined experimental (chick embryo) and computational (finite element modeling) approach, we study the forces driving early s-looping when the primitive ventricle moves to its definitive position inferior to the common atrium. New results from our study indicate that the primitive heart has no intrinsic ability to form an s-loop and that extrinsic forces are necessary to effect early s-looping. They support previous studies that established an important role for cervical flexure in causing early cardiac s-looping. Our results also show that forces applied by the splanchnopleure cannot be ignored during early s-looping and shed light on the role of cardiac jelly. Using available experimental data and computer modeling, we successfully developed and tested a hypothesis for the force mechanisms driving s-loop formation. Forces external to the primitive heart tube are necessary in the later stages of cardiac looping. Experimental and model results support our proposed hypothesis for forces driving early s-looping.

Semaphorin 3d signaling defects are associated with anomalous pulmonary venous connections

Total anomalous pulmonary venous connection (TAPVC) is a potentially lethal congenital disorder that occurs when the pulmonary veins do not connect normally to the left atrium, allowing mixing of pulmonary and systemic blood. In contrast to the extensive knowledge of arterial vascular patterning, little is known about the patterning of veins. Here we show that the secreted guidance molecule semaphorin 3d (Sema3d) is crucial for the normal patterning of pulmonary veins. Prevailing models suggest that TAPVC occurs when the midpharyngeal endothelial strand (MES), the precursor of the common pulmonary vein, does not form at the proper location on the dorsal surface of the embryonic common atrium. However, we found that TAPVC occurs in Sema3d mutant mice despite normal formation of the MES. In these embryos, the maturing pulmonary venous plexus does not anastomose uniquely with the properly formed MES. In the absence of Sema3d, endothelial tubes form in a region that is normally avascular, resulting in aberrant connections. Normally, Sema3d provides a repulsive cue to endothelial cells in this area, establishing a boundary. Sequencing of SEMA3D in individuals with anomalous pulmonary veins identified a phenylalanine-to-leucine substitution that adversely affects SEMA3D function. These results identify Sema3d as a crucial pulmonary venous patterning cue and provide experimental evidence for an alternate developmental model to explain abnormal pulmonary venous connections.

Blood Flukes (Digenea: Aporocotylidae) of Epipelagic Lamniforms: Redescription ofHyperandrotrema cetorhinifrom Basking Shark (Cetorhinus maximus) and Description of a New Congener from Shortfin Mako Shark (Isurus oxyrinchus) off Alabama

We emend the original generic diagnosis for Hyperandrotrema Maillard and Ktari, 1978 , and redescribe its type species Hyperandrotrema cetorhini Maillard and Ktari, 1978 (Digenea: Aporocotylidae Odhner, 1912), based on the holotype and 2 paratypes collected from the heart of basking shark (Cetorhinus maximus). We also describe Hyperandrotrema walterboegeri Orélis-Ribeiro and Bullard n. sp. based on light and scanning electron microscopy of 6 adult specimens collected from the heart of a shortfin mako shark (Isurus oxyrinchus Rafinesque, 1810) captured from Viosca Knoll (29°11.70'N, 88°33.32'W; 123 km southwest of Dauphin Island, Alabama), northern Gulf of Mexico. Hyperandrotrema spp. infect lamniforms and differ from all other nominal aporocotylids at least by having a ventrolateral field of robust C-shaped spines (rather than transverse rows of minute, shaft-like spines), an inverse U-shaped intestine with extremely elongate ceca terminating near the level of the excretory bladder, and a common genital pore that comprises the dorsal opening of a common genital atrium. Adults of the new species exceeded 12 mm in total length, making them the largest of the nominal fish blood flukes. The new species further differs from H. cetorhini by the combination of having an adult body that is 7-8 times longer than wide, large midbody tegumental spines measuring 25-38 μm long × 10-12 μm wide, a long vas deferens 4-5% of the body length, a testis 9-11 times longer than wide, and a large ootype 105-150 μm long × 85-105 μm wide. This is the first report of Hyperandrotrema from the Gulf of Mexico and the second aporocotylid species reported from an epipelagic elasmobranch. Our results demonstrate that ecologically related (epipelagic, marine) and phylogenetically related (Lamniformes) definitive hosts are infected by morphologically similar (congeneric) fish blood flukes.

Advancing Cor Triatriatum in Fontan Circulation

A 14-year-old female with mitral atresia, double outlet right ventricle, l-malposition of the great arteries, pulmonary valvular stenosis, bilateral superior vena cavae, and juxtaposed right atrial appendage had undergone a staged Fontan completion at 22 months of age. Ten years later, transthoracic echocardiography detected blood flow acceleration in the left atrium and scheduled follow-up cardiac catheterization revealed a 3.3 mm Hg pressure gradient between the common pulmonary venous chamber and left atrium, confirming cor triatriatum. An abnormal septum in the left atrium was surgically resected through a left-side right atriotomy. Postoperative echocardiography showed no accelerated blood flow in the left atrium.

Coincidence of Congenital Agenesis of Left Lung and Common Atrium: A Very Rare Case

Pulmonary agenesis is characterized by undeveloped pulmonary vessels, bronchi as well as lung parenchyma and is a rare congenital anomaly with unknown etiology. It is usually diagnosed during childhood. Nearly, one third of these patients have concomitant congenital heart diseases. While more than 50% of these patients die before the age of 5 years, some other patients may be asymptomatic throughout their life. On the other hand, common atrium, another rare congenital anomaly, is characterized by complete absence of the atrial septum and is usually accompanied by atrioventricular valve malformations. An 18-year-old male presented with concomitant pulmonary agenesis and common atrium and had not undergone surgery due to high risk for mortality.

An unusual case of cor triatriatum mimicking mitral stenosis, whilst masking mitral regurgitation

Edwin et al. reported a case of cortriatriatum in the setting of a common atrium and highlight some of the limitations of Transthoracic Echocardiography (TTE) in the process [1]. We present a 62 year old male, who was admitted with worsening dyspnoea (NYHA Class 2) and palpitations. Relevant past medical history includes a previous myocardial infarction, hypercholesterolemia and atrial fibrillation. Coronary angiography revealed three-vessel coronary artery disease on a background of previously diagnosed cortriatriatum sinistrum. The preoperative TTE showed the membrane to be thin and mobile, dividing the left atrium into two chambers. The presence of any fenestrations could not be detected via echocardiography. During the peri-operative period, Transoesophageal Echocardiography (TOE) was used (Fig. 1). Colour doppler ultrasound showed the presence of turbulent flow and revealed that the membrane was mimicking mitral stenosis, as would be expected in such cases, with resulting dilation of the left atrium. However, the mitral valve itself was revealed to be regurgitant. The regurgitant jet was located posteriorly and only ascended for approximately 2 cm before reaching the intra-atrial membrane and being diverted. This finding had not been detected pre-operatively. Additionally, there was Left Ventricular (LV) dilation and reduced LV function from good to moderate. Intraoperatively a small elliptical orifice was revealed and shown to be a continuation of the left atrial appendage. The membrane itself was calcified around the orifice. The membrane was completely excised and the mitral valve exposed to enable repair using an annuloplasty ring. Coronary artery bypass grafts were done to treat the three vessel disease.

Coincidence of Total Anomalous Pulmonary Venous Drainage to the Superior Vena Cava, Common Atrium, and Single Ventricle: A Very Rare Condition

Single ventricle defects, including inflow emptying into 1 ventricle, occur at an incidence of 0.05-0.1 per 10,000 live births. The association of total anomalous pulmonary venous drainage (TAPVD) with single ventricle defects is a risk factor for poor outcome. Conversely, common atrium, a rare congenital anomaly, is an endocardial cushion defect. We present a rare case of an unoperated patient with coincidence of a single ventricle defect, TAPVD with direct drainage from the pulmonary veins into the superior vena cava, and common atrium. To the best of our knowledge, such a case has not been previously reported in the literature.

Novel Fenestration Designs for Controlled Venous Flow Shunting in Failing Fontans With Systemic Venous Hypertension

The Fontan procedure is employed as the final-stage palliation in single-ventricle congenital heart patients and results in diversion of venous blood directly to the pulmonary arteries. Fontan patients have been known to suffer from postoperative systemic venous hypertension, which in turn is associated with pleural effusions and protein losing enteropathy, leading to a decreased duration and quality of life. Despite the ongoing debate on its benefits, a circular fenestration hole (typically 4 mm) establishing a venous shunt to the common atrium is traditionally employed to relieve venous pressure in the Fontan conduit and improve early postoperative Fontan hemodynamics. However, these improvements come at the cost of reduced oxygen saturation due to excessive right-to-left shunting if the fenestration is permanent. The ideal "selective" fenestration would therefore limit or eliminate shunt flow at tolerable systemic venous pressures and allow increased flow at high pressures. The objective of this study is to introduce new fenestration designs that exhibit these desirable pressure-flow characteristics. Novel plus-shaped and S-shaped fenestration designs with leaflets are introduced as alternatives to the traditional circular fenestration, each having identical effective orifice areas at the fully open states. In vitro steady leakage flow tests were performed for physiological flow-driving pressures in order to obtain pressure-drop versus flow-rate characteristics. In addition, the leaflet opening kinematics of the plus-shaped fenestration was investigated computationally using finite element simulation. Fluid-structure interaction analysis was performed to determine leaflet displacements and pressure-flow characteristics at low pressures. Further, a lumped parameter model of the single-ventricle circuit was created to simulate pulsatile flow conditions For the plus-shaped fenestration, the flow rate was found to increase nonlinearly with increased driving systemic venous pressures at high physiological-pressure drops which did not cause the leaflets to fully open, and linearly for low driving pressures. These results indicate that leaflets of the plus-shaped fenestration design activated passively after a critical systemic venous pressure threshold. This feature is ideal for minimizing undesirable excessive venous shunting. A large variability in shunting flow rate may be obtained by changing the shape, thickness, size, and material of the fenestration to suit requirements of the patient, which can further limit shunt flow in a controlled manner.

A new monozoic tapeworm, Lobulovarium longiovatum n. g., n. sp. (Cestoda: Caryophyllidea), from barbs Puntius spp. (Teleostei: Cyprinidae) in the Indomalayan region

A new caryophyllidean cestode is described from barbs Puntius spp. (Cypriniformes: Cyprinidae), with P. sophore (Hamilton) as its type-host, in the Ganges and Brahmaputra river basins in India and Bangladesh, and a new genus, Lobulovarium n. g., is proposed to accommodate it. The genus belongs to the Lytocestidae because its vitelline follicles are situated in the cortex. It is typified by: (i) a peculiar ovary, which is roughly H-shaped, but with asymmetrical, irregular lobes on its ventral and dorsal sides; (ii) an extensive vitellarium formed by numerous vitelline follicles scattered throughout the cortex; (iii) a long, conical postovarian part of the body with numerous vitelline follicles; (iv) a broadly digitate scolex with a slightly protrusible central cone; (v) a single gonopore (male and female genital ducts open via a single pore and a common genital atrium is absent); and (vi) a small number of testes (< 60). Molecular data (partial sequences of the lsrDNA) indicate that Lobulovarium longiovatum n. sp. belongs among the most basal caryophyllidean cestodes, being unrelated to species from siluriform catfishes in the Indomalayan region. Paracaryophyllaeus osteobramensis (Gupta & Sinha, 1984) Hafeezullah, 1993 (syn. Pliovitellaria osteobramensis Gupta & Sinha, 1984) from another cyprinid fish, Osteobrama cotio (Hamilton), in Uttar Pradesh, India, is tentatively transferred to Lobulovarium as L. osteobramense (Gupta & Sinha, 1984) n. comb. It differs from L. longiovatum by having much smaller eggs (length <50 μm versus >90 μm in L. longiovatum), which are spherical (length/width ratio 1:1 versus 2.5-3:1 in the new species), and the presence of vitelline follicles alongside the ovarian lobes (almost completely absent in L. longiovatum).

Divided Left Atrium (Cor Triatriatum) in the Setting of Common Atrium

Cor triatriatum is commonly used to describe the congenital malformation in which an anomalous fibromuscular shelf divides the left atrium. We report the successful surgical repair of an extremely rare combination of divided left atrium, virtual absence of the interatrial septum, and anomalous right pulmonary venous connection in a 7 year-old girl. Preoperative transthoracic echocardiography could not detect the dividing left atrial shelf. In this report, we point out the limitations of transthoracic echocardiography in the diagnosis of atypical cases of so-called cor triatriatum and highlight errors in the scientific literature resulting from the variance in terminology ascribed to congenital division of the left atrium.

Outcome of Left Atrial Isomerism at a Single Institution

Left atrial isomerism includes a complex spectrum of cardiac and extracardiac anomalies. The records of all patients with left isomerism born during the period of 1973-2010 and treated at the Children's Hospital, Helsinki were reviewed. The short- and long-term outcomes were studied. The review included 38 patients (50% females). The overall survival with left atrial isomerism was 63% during a median follow-up time of 16years (range, 4-30years). Extracardiac anomalies were noted in 14 (37%) of 38 cases. Cardiac defects included dextrocardia in 26%, partially or totally anomalous pulmonary venous return in 29%, common atrium in 50%, atrioventriculoseptal defect in 73%, single ventricle in 40%, ventriculoseptal defect without atrioventricular defect in 11%, transposition in 21%, double outlet of the right ventricle in 26%, pulmonary stenosis or atresia in 61%, and left ventricular outflow obstruction in 24% of the cases. Cardiac arrhythmias were presented in 71% and pacemaker treatment in 29% of the cases. Of the 38 patients, 33 had cardiac surgery. Simple palliative methods were used in 11 cases, single-ventricle palliation in 12 cases, and operation with a biventricular track in 10 cases. In the groups that had surgery, 3 of 11 patients, 3 of 12 patients, and 3 of 10 patients died, respectively. In this review, 14 deaths occurred, associated with extracardiac anomalies in five cases and with cardiac arrhythmia in four cases. Five postoperative deaths occurred. At this writing, all three patients who had heart transplantation are alive. Complicated heart defects associated with severe arrhythmias and extracardiac anomalies contribute to a high mortality rate with left isomerism. Cardiac transplantation was considered a good option for selected patients.

An Uncommon Course of the Right Superior Vena Cava in a Patient With Heterotaxy Syndrome

We present the case of an infant with congenital heart disease which includes a partial atrioventricular canal defect with the absence of the atrial septum (common atrium) and an extremely uncommon course of the right-sided superior vena cava (SVC) including an intra-atrial segment coursing intramurally along the right posterolateral atrial wall, with an intracardiac orifice situated low within the right side of the atrium, close to the atrial orifice of the right hepatic veins. This feature of the anatomy was discovered intraoperatively at the time of surgical repair. Systemic venous anatomy also included interrupted inferior vena cava (IVC) with azygos continuation to a left-sided SVC draining directly into the left side of the atrium. The successful surgical procedure included tunneling of the left-sided SVC to the right side of the common atrium and atrial septation with a patch.

A case of single atrium and single ventricle physiology with bilateral cleft lip and palate for lip repair surgery

Bilateral cleft lip and palate may occasionally be associated with complex congenital cyanotic heart disease. An infant with common atrium and single ventricle with infundibular pulmonary stenosis (Blalock-Taussig shunt done recently) presented for lip repair surgery. Balanced general anesthesia was administered using sevoflurane along with a regional nerve block to maintain optimal pulmonary and systemic vascular resistance.

Total Cavopulmonary Connection With Innovative Combined Lateral Tunnel and Extracardiac Conduit in Heterotaxy Syndrome

Total cavopulmonary connection remains a challenging procedure when treating heterotaxy syndrome patients with a widely separate drainage of the inferior vena cava and the hepatic vein into the common atrium. We trimmed a Gore-Tex tube graft (W.L. Gore & Associates, Flagstaff, AZ) to form a skirt to cover both openings of the inferior vena cava and the hepatic vein. This tubular structure pierces the atrium and travels outside the heart, and then reaches the inferior side of the pulmonary artery to avoid pulmonary vein obstruction. We then sutured the pierced atriotomy margin to the conduit. This innovative procedure has been shown to accommodate the widely separated hepatic vein drainage with a promising outcome.