Ventriculoarterial Connections Research Articles

A VERY COMPLEX CASE OF DORV TYPE FALLOT: A CASE REPORT

Abstract Double outlet right ventricle (DORV) is a rare CHD characterized by ventriculoarterial connection in which both great arteries arise entirely or predominantly from the right ventricle. Its prevalence is 0.9% of all CHDs. Several chromosomal abnormalities have been associated with DORV, including trisomy 13, trisomy 18, and chromosome 22q11 deletion. Hearts with DORV are an extremely heterogeneous group showing various morphological features, connections, and relationships at each level of the cardiac segments and intersegmental junctions. As a consequence, the clinical manifestations and required surgical procedures for patients with DORV are variable. The anatomical spectrum is higly variable: it can be associated with ventricular septal defect, transposition of the great arteries (TGA) and functional single ventricle. We report the case of an infant with prenatal diagnosis of CHD and finding partial duplication of the short arm of the X chromosome. Pregnant woman at 22 weeks comes to the outpatient clinic to perform follow–up ultrasound. At that site finding of CHD which was later confirmed at birth and therefore prostaglandin therapy was started. Echocardiogram showed a wide DIV from malalignment with antero–cephalic deviation of the conal septum with significant aortic destroposition configuring picture of double outlet right ventricle. It also presents a complex right efflux obstruction already at subvalvular pulmonary departure. The pulmonary valve presented dysplastic, with en dome motion, hypoplastic. Pulmonary perfusion appears to be provided predominantly by the Botallo‘s duct originating from the isthmic region of the aorta and leading with a long and tortuous course to the pulmonary confluence near the left pulmnary artery with high velocity flow. The remaining findings are normal. Therefore, in view of the clinical picture, he was subjected to palliative surgery of right systemic–pulmonary shunt between the brachiocephalic trunk and right pulmonary artery (modified Blalock–Taussing surgery) in 3.5mm PTFE and ligation of Botallo‘s duct. At subsequent follow–up, the systemic–pulmonary shunt appeared pervious with regular flow. Wide DIV from anterior malalignment with aorta cavalier to DIV. Severe stenosis at complex pulmonary outflow with max gradient up to 124mmHg with dynamic and valvular component. For the subsequent course of treatment, the patient will be discussed collegially.

How best can we name the channels seen in the setting of deficient ventricular septation?

Surgical repair of channels between the ventricles is enhanced when the surgeon knows precisely where to place a patch, or baffle, so as to restore septal integrity. The paediatric cardiologist should provide the necessary information. Communication will be enhanced if the same words are used to account for the structures in question. Currently, however, the same term, namely "ventricular septal defect," is used to account for markedly different areas within the heart. Closure of perimembranous defects found in hearts with concordant or discordant ventriculo-arterial connections restores the integrity of the ventricular septum, at the same time separating the systemic and pulmonary blood streams. When both arterial trunks arise from the right ventricle, in contrast, the surgeon when placing a baffle so as to separate the blood streams, does not close the channel most frequently described as the "ventricular septal defect." In this review, we show that the perimembranous lesions as found in hearts with concordant or discordant ventriculo-arterial connections are the right ventricular entrances to the areas subtended beneath the hinges of the leaflets of the aortic or pulmonary valves. When both arterial trunks arise from the right ventricle, and the channel between the ventricles is directly subaortic, then the channel termed the "ventricular septal defect" is the left ventricular entrance to the comparable space subtended beneath the aortic root. We argue that recognition of these fundamental anatomical differences enhances the appreciation of the underlying morphology of the various lesions that reflect transfer, during cardiac development, of the aortic root from the morphologically right to the morphologically left ventricle.

Double-Outlet Left Ventricle: Case Series and Systematic Review of the Literature.

Double-outlet left ventricle (DOLV) is an abnormal ventriculo-arterial connection characterized by the origin of both great arteries from the morphological left ventricle. The aim of our paper is to describe the morphological and imaging features of DOLV and to assess the prevalence of the associated malformations and their surgical outcomes. METHODS From 2011 to 2022, we retrospectively reviewed the electronic case records of patients diagnosed with DOLV at the Bambino Gesu Children's Hospital. A systematic search was developed in MEDLINE, Web of Science, and EMBASE databases to identify reports assessing the morphology and outcomes of DOLV between 1975 and 2023. RESULTS: Over a median follow-up of 9.9 years (IQR 7.8-11.7 y), four cases of DOLV were identified at our institution. Two patients were diagnosed with (S,D,D) DOLV subaortic VSD and pulmonary stenosis (PS): one patient had (S,D,D) DOLV with doubly committed VSD and hypoplastic right ventricle, and another patient had (S,D,L) DOLV with subaortic VSD and PS (malposition type). Pulmonary stenosis was the most commonly associated lesion (75%). LITERATURE REVIEW: After systematic evaluation, a total of 12 reports fulfilled the eligibility criteria and were included in our analysis. PS or right ventricular outflow tract obstruction was the most commonly associated lesion (69%, 95% CI 62-76%). The most common locations of VSD were subaortic (pooled prevalence: 75%, 95% CI 68-81), subpulmonary (15%, 95% CI 10-21), and doubly committed (7%, 95% CI 4-12). The position of the great arteries showed that d-transposition of the aorta was present in 128 cases (59% 95% CI 42-74), and l-transposition was present in 77 cases (35%, 95% CI 29-43).

Optimizing Surgical Selection for Transposition With Left Ventricular Outflow Tract Obstruction

BackgroundStudies that have assessed the Rastelli and Nikaidoh operations for transposition of the great arteries (TGA) with obstructed left ventricular outflow tract obstruction (LVOTO) have not fully evaluated the anatomic drivers that may contribute to surgical selection. We present our procedural selection process for optimizing outcomes of complex TGA in the modern era. MethodsThis is a single-center, retrospective study that included pediatric patients who underwent either a Nikaidoh or Rastelli operation for the treatment of TGA-LVOTO, congenitally corrected TGA–LVOTO, or double-outlet right ventricle TGA type–LVOTO from June 2004 to June 2021. ResultsThere were 34 patients stratified by Nikaidoh (n = 16) or Rastelli (n = 18) operation. The incidence of all postoperative complications and mortality was low, and the incidence of complications between the groups was similar. Patients were more likely to have undergone a Nikaidoh than a Rastelli if they had a pulmonary annulus >5 mm (87.5% vs 11.1%), anteriorly/posteriorly oriented great vessels (88% vs 8%), remote (80% vs 11%) or restrictive (75% vs 6%) ventricular septal defect, and right ventricular hypoplasia (50% vs 0%; all, P < .05). The resulting rates of reoperation were similar between the groups (44.0% vs 37.5%; P = .24) and largely composed of conduit replacements in the Rastelli patients and valvular repairs or replacements in the Nikaidoh group. Rates of catheter-based interventions were also similar. ConclusionsThese findings suggest that for the optimal treatment of conotruncal anomalies with discordant ventriculoarterial connections, procedural selection should be based on pathoanatomic criteria that can ensure patients undergo the operation most suited to their anatomy.

Double Outlet Right Ventricle with Pulmonary Atresia and Antero-Posterior Malposition of Great Arteries: Transthoracic Echocardiographic Evaluation of a rare cyanotic congenital heart disorder

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle, This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We are reporting a case of seven year old deeply cyanotic child with DORV, subaortic VSD with rare anatomic features of antero-posterior malposition of great arteries (GA) and pulmonary vavular atresia, with conspicuous absence of patent ductusarteriosus (PDA) or major aorto-pulmonary collaterals (MAPCAs), despite the presence of atretic pulmonary valve. Keywords: DORV, Pulmonary atresia, TGA, VSD, subaortic VSD, overriding of Aorta, cyanotic congenital heart defects.

A rare ventriculoarterial connection: double outlet of both ventricles.

Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling.

C15 PERCUTANEOUS EDGE–TO–EDGE REPAIR USING EDWARDS PASCAL SYSTEM FOR SEVERE TRICUSPID REGURGITATION IN CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES

Abstract Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac malformation characterized by ‘double’ discordance of atrioventricular and ventriculo–arterial connections, resulting in a systemic tricuspid atrioventricular valve and a systemic right ventricle. Clinical case We describe a case report of 54 years–old male patient with ccTGA, diagnosed when he was 3 years–old. Patient performed regular follow–up. Since December 2019, the patient reported onset of effort dyspnoea (NYHA III), fatigue and exercise intolerance. Despite medication optimization, his symptoms persisted. He was referred at our Centre for specialist evaluation. Transthoracic echocardiography showed mild dilatation and dysfunction of right ventricle, massive systemic tricuspid valve regurgitation (EROA 42 mm2), due to multiple jets and systolic pulmonary artery pressure of 45 mmHg. As reported by most recent guidelines, indication was given for surgical replacement of the systemic tricuspid valve. However, the patient refused the cardiac surgery option. Transesophageal echocardiography (TEE) screening showed feasibility of percutaneous approach to tricuspid regurgitation. Being the jet central, the strategy was to place one device implant between the septal and the anterior leaflet (9–3 oriented), as central as possible. Following simultaneous leaflet capture and further optimization of leaflet, the result was excellent. Post–implant deployment TEE revealed only mild–to–moderate residual tricuspid regurgitation, with no tricuspid stenosis (mean gradient 2 mm Hg). The result was a percutaneous bicuspidization of a tricuspid systemic valve. The patient recovered quickly and was discharged to home 2 days after the procedure. Patient experienced significant improvement in functional status (NYHA I), exercise capacity and quality of life at 6 months follow–up. Conclusions In ccTGA patients, treatment of severe tricuspid regurgitation and improvement of the systemic ventricle’s systolic function is critical since progression of dysfunction of the RV and tricuspid regurgitation are considered key determinants for morbidity and mortality in these patients. Based on the results obtained in our case, percutaneous edge–to edge repair seems to be a reasonable alternative to the surgical approach and three–dimensional transesophageal echocardiography is imperative for the proper visualization of the leaflets outcome of the procedure and his durability.

1002 A HEART BEYOND A MIRROR: WHEN CCTGA ENCOUNTERS SITUS VISCERUM INVERSUS

Abstract Background Congenitally corrected transposition of great arteries (ccTGA) is an uncommon complex congenital heart disease with atrio-ventricular and ventriculo-arterial connections discondance. ccTGA may be associated with a situs solitus or situs inversus (34% of cases). Situs inversus is a mirror image of normal with the systemic ventricle situated on the right side. Instead, dextrocardia represents 20% of cases. Case clinic and discussion Came to our observation a 61 years old female, symptomatic for dyspnea on exertion (NYHA II). She had no past medical history of cardiovascular events. In anamnesis two full-term pregnancies without complications. Transthoracic echocardiogram found atrioventricular and ventriculo-arterial discordance in absence of significant valvulopathy. Cardiac computed tomography showed pulmonary veins linked to right atrium, superior and inferior cava veins connected to the left atrium; right atrium with tricuspid valve was connected to a morphologically left ventricle and left atrium with mitral valve was linked to morphologically right ventricle; pulmonary artery was connected to morphologically left ventricle instead aorta with aortic valve was linked to morphologically right ventricle. Cardiac MRI confirmed cctga in situs viscerum inversus, mild subpulmonary stenosis, moderate dilatation of arterial pulmonary trunk, and also intramyocardial late gadolinium enhancement due to fibrosis involving anterior and inferior interventricular junctions. Cardiac Holter monitoring showed sinus rhythm with some brief phases of low atrial rhythm, monomorphic isolated ventricular extrasystoles in absence of significant hyperkinetic or hypokinetic arrhythmias. CcTGA represents approximately 0.5% of all congenital heart disease. If undiagnosed in childhood, people usually become symptomatic during the first decades of life. Dyspnea, syncope and fatigue are the most frequent symptoms detected. Cardiac conduction disorders such as atrioventricular blocks are common due to the abnormal development of cardiac structures. Quality of life and its expectancy are related to the latency of the onset of heart failure symptoms. Only few patients remain asymptomatic beyond 50 years old. Symptoms and signs are frequently due to right sided (systemic) heart dysfunction and tricuspid valve insufficiency. A particular clinical situation worthy of attention is pregnancy because of the hemodynamic imbalance occurring. In fact, cardiac output increases of 40–50% above baseline determining an augmentation of stroke volume and heart rate. For these reasons, echo surveillance is needed every 4-8 weeks because of the increased risk of acute heart failure. An accurate assessment of heart rhythm has to be done due to the known predisposition to bradyarrhythmic and tachyarrhythmic events in ccTGA. Conclusions CcTGA patients require a strict cardiological follow up with echocardiographic assessment and periodic heart rhythm monitoring, in order to early detect worsening of cardiac function and significant abnormalities of the rhythm.

Long-term results after the REV (réparation à l’étage ventriculaire) procedure for malposition of the great arteries

Several surgical techniques can be used to repair anomalies of the ventriculoarterial connection with sub-pulmonary stenosis. Although there is no equivocal evidence that one technique is superior to another, the arterial switch operation (ASO) and Rastelli procedure are the preferred options to correct transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO). Despite innovative features and satisfactory short- and long-term results, the “réparation à l’étage ventriculaire” (REV) procedure has not gained large popularity and still struggles to establish itself as a legitimate alternative to the Rastelli operation. By describing our long-term results, we wish to address the misconceptions about the technical aspects and the long-term results of this technique that have been suggested to explain the reluctance towards it. Between 1980 and 2022, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, VSD and LVOTO ( n = 113, 72.0%). Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) had a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 65 patients (41.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89%. Thirty-seven patients (23.6%) required 67 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months – 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6% respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years ( Fig. 1 ). The REV procedure is a good alternative to the Rastelli intervention when an arterial switch operation is not feasible. A quarter of the patients required redo surgery on the right ventricular outflow tract. Reoperations for left ventricular outflow tract obstruction are scarce.

A Rare Shunt

A 12-DAY-OLD girl (weight: 3 kg; height: 50 cm) presented to the authors’ institution in respiratory distress. The child was tachypneic (70 breaths/min), with an arterial oxygen saturation of about 65%-to- 70% on 3 L/min oxygen flow through a nasal cannula, and had signs of severe heart failure (Fig 1). Transthoracic echocardiography revealed situs solitus, levocardia, atrioventricular and ventriculoarterial concordant connections, normal systemic and pulmonary venous drainage, membranous pulmonary atresia, and an intact ventricular septum.

Единственный желудочек сердца у кота породы мейн-кун

Univentricular heart (UH) is a rare group of congenital heart disease characterized by the atrial chambers connects with one ventricular chamber. The most frequent UH is double inlet morphologically left ventricle with the discordant ventriculo-arterial connection. The main diagnostic methods are echocardiography, angiocardiography and magnetic resonance imaging. In humans, heart transplantation is a radical method of surgical treatment. Palliative surgical management is different based on the underlying anatomy and include 2 or 3 stage defect correction with the exclusion of the rudimentary ventricle from the circulation. The focus of therapy is to management heart failure and pulmonary hypertension. The article describes the stages of diagnosis and treatment of UH in a Maine Coon.

Transposition physiology in the setting of concordant ventriculo-arterial connections.

To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re-interventions, for the rare instances of transposition physiology with concordant ventriculo-arterial connections. We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo-arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Among reported cases, just over two-thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four-fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one-fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multistaged. Overall mortality was 25%, with one-fifth of patients requiring pacemakers for surgically-induced heart block. The majority of survivors were in good functional state. The flow patterns produced by discordant atrioventricular and concordant ventriculo-arterial connections remain an important, albeit rare, indication for atrial redirection or hemi-Mustard's procedure with bidirectional Glenn. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long-term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits.

Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers on multidetector CT.

To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector computed tomography (CT) angiography. We retrospectively evaluated all the CT angiography studies performed for the evaluation of various congenital heart diseases at our center from January 2014 to December 2021. Cases with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers were identified. The types of cardiovascular involvement as identifiable on CT angiography were studied and compiled. A total of 38 patients with left atrioventricular valve atresia and patent aortic root were included (median age: 5 months, males: 31). A total of 89.5% had usual atrial arrangement and 10.5% had mirror imagery of atria. Normal drainage of right and left superior caval veins and inferior caval vein into systemic venous atrium was seen in 77.8%, 61.5%, and 86.6%, respectively. Anomalous pulmonary venous drainage was seen in 3 (7.9%) patients. Atrial and ventricular septal defects were the most common associations. Imperforate left atrioventricular membrane was seen in 9 (23.7%) patients and absent left atrioventricular connection in 27 (71.1%). Discordant right atrioventricular connection was seen in 5 (13.1%) patients. While a double outlet right ventricle (76.3%) was the most common ventriculoarterial connection, discordant ventriculo-arterial connection (transposition) in 4 (10.5%). Most patients had good sized pulmonary arteries. Patent arterial duct and right aortic arch were seen in 29% and 21%, respectively. Aberrant right subclavian artery was the most common arch vessel anomaly. Coronary anomalies were seen in 7 patients (19%); single coronary artery being the most common. Majority of the patients with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers have usual atrial arrangement, normal right atrioventricular connections and a double outlet configuration of the right ventricle. Absent left atrioventricular connection is a more common than imperforate left atrioventricular valve membrane. Atrial and ventricular septal defects are the most common associations. Comprehensive CT based evaluation of vascular and nonvascular thoracic structures is imperative before surgery or interventions in patients with mitral atresia.

Juxtaposition of atrial appendages: Evaluation of cardiovascular morphology and associated anomalies on multidetector computed tomography angiography.

To evaluate the morphology and associated cardiovascular abnormalities in patients with juxtaposition of atrial appendages (JAA) on multidetector computed tomography (MDCT) angiography. We performed the retrospective study at a tertiary referral center to identify patients diagnosed with JAA on MDCT angiography using dual-source CT scanner between January 2014 and April 2021. The various imaging abnormalities evaluated included the type of JAA, morphological and positional classification of JAA, atrial situs, cardiac position, atrioventricular and ventriculoarterial connections, great vessel relationship, and other associated cardiovascular anomalies. We identified 129 patients with JAA. Left-sided juxtaposition was seen in 124 (96.1%) patients while right-sided juxtaposition was seen in 5 (3.9%) patients. Among patients with left-sided juxtaposition, frequent cardiovascular associations included outflow tract malformations (100%), hypoplastic right ventricle (40.3%), tricuspid atresia (32.2%), and right-sided heart (19.4%). The most frequent outflow tract malformation was double outlet right ventricle (DORV; 60.5%) followed by transposition of great arteries (20.1%) and transposed aorta with pulmonary atresia (18.5%). In patients with right-sided juxtaposition, outflow tract malformation was seen in three (60%) patients with one (20%) patient each showing DORV, transposed aorta with pulmonary atresia, and tetralogy of Fallot, respectively. The identification of JAA on MDCT angiography suggests coexistence of various complex congenital heart diseases, especially outflow tract malformations. A meticulous search is imperative to identify this anomaly before interventional or surgical procedures to avoid complications.

Deep Phenotypic Analysis for Transposition of the Great Arteries and Prognosis Implication

BackgroundTransposition of the great arteries (TGA) consists of about 3% of all congenital heart diseases and 20% of cyanotic congenital heart diseases. It is always accompanied by a series of other cardiac malformations that affect the surgical intervention strategy as well as prognosis. In this study, we comprehensively analyzed the phenotypes of the patients who had TGA with concordant atrioventricular and discordant ventriculoarterial connections and explored their association with prognosis.Methods and ResultsWe retrospectively reviewed 666 patients with a diagnosis of TGA with concordant atrioventricular and discordant ventriculoarterial connections in Fuwai Hospital from 1997 to 2019. Under the guidance of the Human Phenotype Ontology database, patients were classified into 3 clusters. The Kaplan‐Meier method was used to analyze the prognosis, and the Cox proportional regression model was used to investigate the risk factors. In this 666‐patient TGA cohort, the overall 5‐year survival rate was 94.70% (92.95%–96.49%). Three clusters with distinct phenotypes were obtained by the Human Phenotype Ontology database. Kaplan‐Meier analysis revealed a significant difference in freedom from reintervention among 3 clusters (P<0.001). To eliminate the effect of surgeries, we analyzed patients who only received an arterial switch operation and still found a significant difference in reintervention (P=0.019).ConclusionsWe delineated a big cardiovascular phenotypic profile of an unprecedentedly large TGA cohort and successfully risk stratified them to reveal prognostic significance. Also, we reported the outcomes of a large TGA population in China.

The VE/VCO2 slope: A useful tool to evaluate the physiological status of children with congenital heart disease

Cardio-pulmonary exercise test (CPET) is becoming a key examination to assess physical capacity and disease severity in paediatric cardiology. The VE/VCO 2 slope has been increasingly used as a surrogate marker for morbidity and mortality in adult heart failure, pulmonary arterial hypertension and for adult patients with CHD. Nevertheless, the use of the VE/VCO 2 slope in children remains limited in the absence of reference values and clearly identified clinical determinants. This study aimed to compare the VE/VCO 2 slope in a paediatric cohort with CHD to that of age- and gender-adjusted healthy controls. We also intended to identify the clinical and CPET variables associated with VE/VCO 2 slope in this population. This cross-sectional study was carried out between November 2010 and September 2015 in two tertiary care paediatric cardiology reference centres. A total of 700 children were enrolled (399 CHD and 301 healthy controls). The mean VE/VCO 2 slope was significantly higher in CHD than in controls (31.6 ± 4.8 vs. 29.3 ± 4.8; P < 0.001). When considering CHD sub-groups, the VE/VCO 2 slope was impaired in single ventricle, complex anomalies of ventriculo-arterial connections, anomalies of the atrioventricular junctions and valves. The VE/VCO 2 slope according to the CHD physiological status was illustrated in the Fig. 1 , it was higher in children with significant pulmonary regurgitation, tricuspid regurgitation, right ventricular hypertension and right ventricle outflow tract (RVOT) obstacle. In the CHD group, VE/VCO 2 slope increase was associated with BMI, the presence of a RVOT obstacle, the number of cardiac catheter procedures, as well as low age, FVC, tidal volume, and PetCO2 ( Fig. 1 ). Increased VE/VCO 2 slope was predominantly in children with single ventricle and/or residual right heart abnormalities suggesting that maldistribution of pulmonary blood flow during exercise is an important CHD-unique determinant of VE/VCO 2 slope.

Third Atrial Chamber

A 10-MONTH-OLD BOY (weight: 5.8 kg; height: 66 cm; body mass index: 13) was taken to the cardiac catheterization laboratory before the creation of a bidirectional Glenn shunt. At 4 days of age, transthoracic echocardiography done for the evaluation of persistent cyanosis revealed heterotaxy (left atrial isomerism), situs ambiguous with levocardia, bilateral superior vena cava, atrial septal defect (9 mm), partial anomalous right pulmonary venous return to the morphologic right atrium, discordant atrioventricular connection and concordant ventriculoarterial connection, with a hypoplastic right ventricle. The child underwent stenting of the patent ductus arteriosus and balloon atrial septostomy on the 11th day of life. Pulmonary artery banding was done at the age of 1 month. During the current admission, a computerized tomography scan revealed the complex connections of the great vessels (Fig 1). A 4-chamber transesophageal echocardiography view displayed the morphologic left atrium, which was on the right side, and multiple coronary artery fistulae opening into an enlarged chamber located superiorly (Fig 2; Video 1). The atrial septal defect could be visualized in the bicaval view (Fig 3; Video 2). A modified midesophageal 4-chamber view displayed a large superior chamber, as well as the atrial septal defect (Fig 4). Agitated saline was injected into a right upper extremity vein. The agitated saline appeared first in the right internal jugular vein and then appeared in the upper atrial chamber, right side atrium (morphologic left atrium), followed by an appearance in the right ventricle (Fig 5, A-D; Video 3). Slight retraction of the probe from the 4-chamber view displayed a right superior vena cava on the right side that was moving into close juxtaposition with the morphologic left atrium (Fig 6; Video 4). Correlation with computerized tomography findings revealed the identity of the structure seen by transesophageal echocardiography into which the right superior vena cava and the coronary artery fistulae were draining. What is the diagnosis? Fig. 2(A) Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with (B) color Doppler displaying an enlarged chamber (white query sign) with multiple coronary artery fistulae opening into the chamber. mLA, morphologic left atrium; RV, right ventricle; TV, tricuspid valve View Large Image Figure Viewer Download Hi-res image Fig. 3Bicaval midesophageal transesophageal echocardiography view displaying the atrial septal defect. View Large Image Figure Viewer Download Hi-res image Fig. 4Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image with color-flow Doppler displaying the atrial septal defect and a large upper chamber marked by white query sign. View Large Image Figure Viewer Download Hi-res image Fig. 5Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying (A) the agitated saline echo contrast injected into an upper limb vein entering the right superior vena cava, (B) the coronary sinus and the morphologic right atrium on the right side, (C) the morphologic left atrium on the right side, and (D) the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 6Midesophageal 4-chamber 2-dimensional transesophageal echocardiography image displaying the right superior vena cava and morphologic right atrium in close proximity. ASD, atrial septal defect; LAA, morphologic left atrial appendage; mLeft atrium, morphologic left atrium; mR, morphologic right atrium; RSVC, right superior vena cava; RV, right ventricle; TV, tricuspid valve. View Large Image Figure Viewer Download Hi-res image

Low discontinuity of care rate for adults with complex congenital heart disease: A single center's experience

BackgroundDiscontinuity of care between childhood and adulthood is an identified problem for those with congenital heart disease. ObjectiveThis study investigated our center's discontinuity of care rate during this transition period. MethodsThe study cohort included those post-Fontan performed in childhood; those post-pulmonary valve, mitral valve, or aortic valve replacements initially performed between birth and age 17 years; those post-atrial switch operations; and those with biventricular hearts and discordance of atrioventricular and ventriculoarterial connections. We included patients born between 1978 and 2000 and cared for at our center initially from a prenatal diagnosis, birth, infancy, or childhood. We defined discontinuity of care as lack of continuing care until a minimum of 20 years of age, after excluding those that died before 20 years old or had documented continuing care at another center. We also tabulated patients that underwent at least one adult invasive procedure during continuous care. ResultsWe identified 245 patients, of which 19 (7.8%) experienced discontinuity of care. Of the 226 in continuous care, 137 (60.6%) underwent 187 invasive procedures as adults: 36 cardiovascular surgeries, 82 surveillance cardiac catheterizations with or without transvenous liver biopsy, 32 interventional cardiac catheterizations, and 37 electrophysiology procedures with or without a pacemaker procedure. ConclusionWe found a low, 7.8% discontinuity of care rate for adults with complex congenital heart disease at our center. Further, for adults with complex congenital heart disease, most required additional invasive procedures as adults.

Fetal heterotaxy: Should we still categorize?

Heterotaxy (HTX) is usually stratified in right (RI) and left isomerism (LI). We analyzed 61 fetal specimens with HTX divided in 5 groups: classic LI [CLI: bronchopulmonary LI, bilaterally absent pectinate muscles (PM), polysplenia, n = 22], non-classic LI (NCLI: one discordant feature, n = 13), classic RI (CRI: bronchopulmonary RI, bilateral extent of PM to the crux, asplenia, n = 12), non-classic RI (NCRI: one discordant feature, n = 6, totally discordant features, n = 8). Non-classic patterns were found in 44% (33% RI, 37% LI). Among those, the status of the spleen was highly variable (NCLI with asplenia 38%, NCRI with single spleen 66%), while the bronchial status was almost always concordant. Intestinal malrotation was present in 83% CRI, 64% CLI, 50% NCLI, 50% NCRI. Extent of PM was highly variable in NCLI (bilaterally left 46%, normal 46%, bilaterally right 8%), and bilaterally right in 83% of NCRI only. Interrupted inferior caval vein was seen in LI only; total extracardiac anomalous pulmonary venous connection (APVC) was seen only in RI, ipsilateral APVC only in LI. A common atrioventricular (AV) junction was constant in RI vs. 66% in LI, with a complete AV canal in 100% RI, 75% LI. Functionally univentricular hearts were found in 67% RI, 46% LI, right in 62.5%. Ventriculo-arterial (VA) connections were abnormal in 100% RI, 59% LI, double outlet right ventricle (DORV) being the most frequent type in all categories. Pulmonary atresia and stenosis were frequent in RI (77%), rare in LI. Aortic arch hypoplasia and coarctation were found almost exclusively in LI (57%). Right-sided aortic arch was found in 67% RI, 6% LI. HTX and isomerism are not synonymous. Extent of PM is not always symmetrical (15%) and cannot therefore be considered the hallmark of HTX. Because HTX includes both abnormal symmetry and random organization of organs, each anatomic feature should be analysed individually rather than trying to categorize.

Multiple Color Doppler Flow Jets Into The Atria in a Child With Heterotaxy Syndrome

A nine-month-old girl (weight: 7.92 kg; height: 70 cm) was taken to the operating room for the creation of a bilateral bidirectional Glenn shunt and atrial septectomy. Patent ductus arteriosus stenting had been done when the child was four days old. Transthoracic echocardiography showed heterotaxy (left atrial isomerism) with situs inversus, discordant atrioventricular and concordant ventriculoarterial connections (ventricular ‘noninversion’), a large ventricular septal defect, membranous pulmonary atresia with a hypoplastic main pulmonary artery, confluent branch pulmonary arteries, aorta anterior and to the right of the pulmonary artery, and bilateral superior vena cava with no bridging vein. The left superior vena cava was draining into the left-sided atrium (morphologic right atrium), while the right superior vena cava was draining into the same atrium via a massively dilated coronary sinus. The inferior vena cava was interrupted, with hemzygous continuation to the left superior vena cava. A computerized tomography scan revealed a right-sided stomach with a midline liver, and demonstrated the complex vascular communications (Fig 1, A and B). Transesophageal echocardiography confirmed the preoperative diagnosis (Fig. 2, Fig. 3; Videos 1 and 2). Color- flow Doppler mapping in the mid-esophageal view demonstrated multiple jets into both the atria and the right-sided ventricle (Fig 4, A and B; Videos 3 and 4). Pulse-wave Doppler of a dominant jet revealed a biphasic flow (Fig 5; Video 5). The intramyocardial origin of the jets was traced toward the aorta (Fig 6; Video 6). Fig. 2The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map showing demonstrating the anatomic abnormality in the child. View Large Image Figure Viewer Download Hi-res image Fig. 3The midesophageal, four-chamber transesophageal echocardiography view with color- flow Doppler map demonstrating the left superior vena cava. Left SVC, left superior vena cava. View Large Image Figure Viewer Download Hi-res image Fig. 4(A) The midesophageal transesophageal echocardiography views with color-flow Doppler map demonstrating jets (white arrows) into the left atrium. (B) The right atrium and the right ventricle. View Large Image Figure Viewer Download Hi-res image Fig. 5The midesophageal pulse-wave Doppler signal interrogation of the jet by transesophageal echocardiography. View Large Image Figure Viewer Download Hi-res image Fig. 6The midesophageal transesophageal echocardiography view displaying the color-flow Doppler mapping delineating the origin of the jet (white arrows). View Large Image Figure Viewer Download Hi-res image