Subpulmonary Stenosis Research Articles

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.

Unusual presentation of a patent ductus arteriosus: A case report

A 48 years old patient, with no medical history was referred to the cardiovascular surgeon of our department for a right medio-ventricular resection diagnosed after 2 episodes of syncope. The transthoracic echocardiography revealed a severe RV hypertrophy with aspect of sub-pulmonary stenosis. An invasive hemodynamic assessment was performed and concluded to a severe Pulmonary arterial hypertension. During the invasive right cardiac exploration, the catheter run easily into an unknown large patent ductus arteriosus (PDA). PDA in adults is an extremely rare clinical phenomenon since it is usually discovered and treated during childhood. However, physicians need to be aware of potential situations, signs and symptoms that might suggest a previously undiagnosed PDA.

A Case of An Unrepaired Tetralogy of Fallot

In most cases, the combination of a ventricular septal defect (VSD), overriding aorta, right ventricular outflow tract obstruction, and right ventricular hypertrophy (collectively known as tetralogy of Fallot [TOF]) will result in cyanosis unless surgically repaired in early infancy. If left uncorrected, around 75% of patients will not reach the age of 10. However, there are a few reported cases of individuals reaching adulthood without surgical correction of these abnormalities. Interestingly, there are key cardiac adaptations by the body that may occur during the progression of TOF, that may allow for well-balanced hemodynamic control that would otherwise not exist. Ultimately, this will minimize cyanosis by balancing blood flow through the pulmonary and systemic circulations. Some of these cardiac adaptations may include left ventricular hypertrophy, delayed progression of any subpulmonic stenosis, and/or the development of aortopulmonary collaterals. While TOF repair is preferably performed during early infancy, repair in adulthood is possible although it does present with increased risk. Additionally, TOF may present with other cardiac abnormalities including atrial septal defects, other VSDs, right aortic arch, anomalous origination of the coronary arteries, etc. Therefore, it is important to sonographically assess for any other cardiac abnormalities in addition to the main findings especially in cases of adult unrepaired TOF.

Sub‐pulmonic stenosis caused by a right ventricular outflow tract vegetation in a children with restricted ventricular septal defect

Infective endocarditis involving the right heart is rarely observed in the pediatric population. Echocardiography plays an important role in its diagnosis, and surgery is indicated in patients with heart failure and persistent sepsis not responding to medical treatment. Here, we report a rare case of restricted ventricular septal defect complicated by a vegetation developed in the right ventricular outflow tract and causing sub-pulmonic stenosis in a 3-year-old male child.

Multimodality Imaging of Transposition of the Great Arteries.

Transposition of the great arteries (TGA) is a congenital conotruncal abnormality characterized by discordant connections between the ventricles and great arteries, with the aorta originating from the right ventricle (RV), and the pulmonary artery (PA) originating from the left ventricle (LV). The two main types of TGA are complete transposition or dextro-transposition of the great arteries (D-TGA), commonly referred to as d-loop, and congenitally corrected transposition (CCTGA), commonly referred to as l-loop or L-TGA. In D-TGA, the connections between the ventricles and atria are concordant, whereas in CCTGA they are discordant, with the left atrium connected to the RV, and the right atrium connected to the LV. D-TGA manifests during the neonatal period and can be surgically managed by atrial switch operation (AtrSO), arterial switch operation (ASO), Rastelli procedure, or Nikaidoh procedure. Arrhythmia, systemic ventricular dysfunction, baffle stenosis, and baffle leak are the common complications of AtrSO, whereas supravalvular pulmonary or branch PA stenosis, neoaortic dilatation, and coronary artery narrowing are the common complications of ASO. CCTGA may manifest late in life, even in adulthood. Surgeries for associated lesions such as tricuspid regurgitation, subpulmonic stenosis, and ventricular septal defect may be performed. A double-switch operation that includes both the atrial and arterial switch operations constitutes anatomic correction for CCTGA. Imaging plays an important role in the evaluation of TGA, both before and after surgery, for helping define the anatomy, quantify hemodynamics, and evaluate complications. Transthoracic echocardiography is the first-line imaging modality for presurgical planning in children with TGA. MRI provides comprehensive morphologic and functional information, particularly in adults after surgery. CT is performed when MRI is contraindicated or expected to generate artifacts. The authors review the imaging appearances of TGA, with a focus on pre- and postsurgical imaging. Online supplemental material is available for this article. ©RSNA, 2021.

Exercise cardiac magnetic resonance imaging to assess dynamic right ventricular outflow tract obstruction in congenital heart disease: a case report.

BackgroundRight ventricular outflow tract obstruction in patients with congenital heart disease is usually assessed using echocardiographic peak instantaneous gradient at rest. Since right ventricular outflow tract obstruction may change during exercise (dynamic right ventricular outflow tract obstruction), we present a case emphasizing the potential use of exercise cardiac magnetic resonance imaging (CMR).Case summaryWe discuss a 15-year-old patient with repaired mid-ventricular sub-pulmonary stenosis type double-chambered right ventricle causing right ventricular outflow tract obstruction and symptoms on exertion. In this case, exercise CMR imaging provided additional information, allowing adequate surgical planning.DiscussionThe additional value of exercise CMR imaging in a case of right ventricular outflow tract obstruction was described. Although exercise cardiac magnetic resonance imaging did not show a significant increase in peak gradient across the right ventricular outflow tract obstruction, shifting and D-shaping of the interventricular septum with subsequent insufficient left ventricular filling (preload) was observed in the patient with recurrent double-chambered right ventricle. This case demonstrates how exercise CMR imaging can be helpful in the clinical decision beyond standard echocardiographic evaluation by providing additional evidence of adverse haemodynamics during exercise.

Abstract 14744: Native Bicuspid Pulmonary Valve in D-loop Transposition of the Great Arteries: Outcomes of the Neo-aortic Valve Function and Root Dilation After Arterial Switch Operation

Introduction: Neo-aortic root dilation (ARD) is common after arterial switch operation (ASO) for D-loop transposition of the great arteries (TGA). We sought to compare short and long-term outcomes for bicuspid native pulmonary valve (BNPV) patients to those with normal trileaflet variants (TNPV). Methods: A retrospective cohort of TGA patients undergoing ASO at Boston Children’s Hospital from 1989-2018 was analyzed, matching BNPV patients 1:3 with TNPV patients by year of ASO; those with >mild subpulmonary stenosis or complex TGA were excluded. Categorical and continuous variables were compared using Fisher’s exact and Wilcoxon rank sum tests, respectively. Kaplan-Meier analyses with log-rank test compared groups for time to first reoperation on the neo-aortic valve, first occurrence of ≥moderate neo-aortic regurgitation (AR), and ARD defined as root z-score ≥4. Hazard ratios were estimated based on the Cox proportional hazards model. Results: A total of 83 BNPV patients were matched with 217 TNPV. BNPV patients were more likely to have a VSD (75% vs 44%, p <0.001). Early surgical outcomes including hospital LOS (11 vs 10 days) and 30-day mortality (3.6% vs 2.8%) were similar. During median 10 years follow-up, neo-aortic valve reoperation occurred in 4 BNPV (6%) vs 6 TNPV (3%) patients, with no statistically significant difference in time to reoperation. More BNPV patients had AR at discharge (4.9% vs 0%, p=0.014) and during follow-up (13.4% vs 4.3%, HR 3.9, p=0.004), with shorter time to first occurrence of AR (Figure 1A); this remained significant after adjusting for presence of VSD. Similarly, ARD was more common in BNPV (45% vs 37%, HR 1.64, p=0.02) with shorter time to first occurrence (Figure 1B). Conclusions: While patients with BNPV have similar short-term ASO outcomes, AR and ARD occur more frequently and earlier compared with TNPV patients. Further long-term studies are needed to determine whether this results in greater need for neo-aortic valve reoperation.

The Fate of Congenitally Corrected Transposition of the Great Arteries Unoperated Before Adulthood

The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear. From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY). At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure. PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes.

Damus-Kay-Stansel procedure with ventricular septal defect enlargement.

A full-term infant who had tricuspid atresia with transposed great arteries, a ventricular septal defect, subpulmonary stenosis with posterior malalignment of the conus septum, bicuspid pulmonary valve, and a high-takeoff left coronary artery was referred to our institution. The subpulmonary stenosis gradually progressed and cyanosis worsened. We successfully performed a Damus-Kay-Stansel procedure and a bidirectional Glenn shunt concomitant with ventricular septal defect enlargement. The conus septum was resected along with thick fibrous tissue through both semilunar valves (without ventriculotomy). Postoperative echocardiography demonstrated that both the ventricular septal defect and the subpulmonary space were enlarged effectively without semilunar valve regurgitation.

Right ventricular outflow tract obstruction caused by a right coronary cusp prolapse: An unusual finding

Patients with large sub-pulmonic ventricular septal defect (VSD) present early as a results of their complications. Some present late, due to the restriction of VSD by the right coronary cusp (RCC) due to its prolapse. In this report, we present a rare case of sub-pulmonic VSD in a 33-year-old man who developed a sub-pulmonic stenosis due to the prolapse of the RCC into the right ventricular outflow tract.

Heterotaxy Syndrome in a Pediatric Cardiac Patient: A Case Report of Rare, Complex Congenital Heart Defects

BackgroundHeterotaxy syndrome, or situs ambiguous, is a multitude of thoracoabdominal congenital irregularities involving organ rearrangements across the body axis. The array of symptoms can be subdivided into either left or right isomerism, which conventionally refers to the anatomy of the designated side presenting bilaterally. While there have been approximately 266 case reports published on heterotaxy syndrome to date, no case report has documented the combination of symptoms seen in our patient, who presents with defects characteristic of both left and right isomerism.PresentationA male infant was delivered early at gestational age 37 weeks and 3 days, due to poor fetal growth. Fetal echocardiogram had been performed prior to delivery and was indicative of complex congenital heart defects, specifically heterotaxy syndrome with dextrocardia, double‐outlet right ventricle with transposed great arteries, a complete atrioventricular canal defect, a large ventricular septal defect and a large common atrium. The suspected defects were confirmed after delivery via echocardiography and cardiac catheterization. Given that heterotaxy can occur by itself or as a secondary feature of another genetic disorder, karyotyping via chromosomal array and chromosomal analysis were performed; however, no chromosomal or genetic abnormalities were identified.Results – Anatomical Features ‐Mirror image dextrocardia with the inferior vena cava and descending aorta located to the left of the spine at the level of the diaphragm ‐There is both a large septum primum and septum secundum defect, which gives rise to essentially a single common atrium, with unrestricted flow across the atrium. The anatomic right atrium is on the patient left, and the anatomic left atrium is on the patient right ‐There are bilateral superior vena cava draining into the anatomic right side of the common atrium is on the patient left. All four pulmonary veins drain into the anatomic left atrium on the patient right. There is also anomalous drainage of the inferior vena cava into the anatomic left atrium is on the patient right ‐The anatomic right ventricular on the patient left gives rise to both the aorta and main pulmonary artery – double‐outlet right ventricle. There is also is transposition of the aorta and main pulmonary artery ‐There is a complete atrioventricular defect with a single common atrioventricular valve with few chordae tendinae attached to the ventricular septum ‐There is severe pulmonary valve stenosis with no appreciable regurgitation. There is no aortic valve stenosis or regurgitation, and the aortic valve is appropriately tri‐leafletConclusionThis form of heterotaxy syndrome partially matches both right and left atrial isomerism. Double‐outlet right ventricle, malposition of the great vessels, and pulmonary and subpulmonary stenosis are typical of right atrial isomerism. In contrast, the common atrioventricular canal defect, bilateral superior vena cava, and anomalous drainage of the inferior vena cava hint at left atrial isomerism. This anatomical case reports highlights the complexity of heart development and highlights the complexity of surgical and long‐term clinical management of heterotaxy syndrome.Cardiac catheterization visualization of dextrocardia and bilateral superior vena cava draining into patients left‐side of common atriumFigure 1Echocardiography visualization of common atrium with small intact septum (yellow arrow), common atrioventricular valve (red arrow), and ventricular septal defect (purple asterisk)Figure 2

P719 Complex cyanotic congenital heart disease and its complications- it is never too late to treat

Abstract Noncardiac complications significantly contribute to the morbidity and mortality of adults with congenital heart disease (CHD), mainly because life expectancy and quality of life for those born with CHD have greatly improved in the last decades. Double-outlet right ventricle (DORV) with a subaortic ventricular septal defect (VSD) associated with subpulmonary stenosis is a complex cyanotic congenital heart disease from the spectrum of tetralogy of Fallot. We present the case of a young male who was diagnosed at the age of 18 with DORV with subaortic VSD (Figure A), subpulmonary stenosis (Figure C) and mitral valve malformation (anterior leaflet cleft (Figure B) and both leaflets prolapse) with secondary moderate mitral regurgitation (MR), associated with major aortopulmonary collaterals, without pulmonary hypertension. There was no surgical correction at that moment. At the age of 26 he presented with recurrent hemoptysis and embolisation of bronchial arteries was performed. In the same year, a cerebral MRI showed signs of multiple infratentorial and supratentorial ischemic strokes. One year later, he was diagnosed with brain abscess and otomastoiditis that were surgically managed; multiple microorganisms were isolated from the two sources (M. morganii, K. pneumoniae, P . aeruginosa) and the patient received prolonged antibiotic therapy. Two months later he presented with clinical, biological and echocardiographic signs of infective endocarditis (IE) and blood cultures confirmed the diagnosis of Candida albicans associated mitral valve IE (Figure D, E, F, G). Antifungal therapy was administered for one month, with the complete disappearance of the vegetation, but worsening of the mitral regurgitation and moderate left ventricular systolic dysfunction. Thus, the patient had a clear indication for mitral valve replacement together with complete repair of the cardiac malformation that seemed still feasible as the pulmonary arteries were well developed, there was no pulmonary vascular disease and the systolic function of the RV was normal. Correction of CHD was performed in March 2019 consisting of repair of the DORV (Figure H), mitral valve replacement (33 mm bileaflet mechanical valve) (Figure I), tricuspid valve repair with RVOT remodelling. Postoperative, the patient clinical status improved significantly and transthoracic echocardiography revealed a mild residual subpulmonary stenosis (Figure J) and normally functioning mitral prosthesis. There are frequent, various complications in the natural history of congenital heart diseases, especially unrepaired. This case illustrates how vicious the circle of complications can get in a case of unrepaired cyanotic cardiac malformation and the difficulty of breaking this circle. The correction of CHD is the best prevention method and should be performed whenever it is feasible, even at an adult age. Abstract P719 Figure.

P1691 A case of congenital pulmonary stenosis

Abstract Introduction We present an unusual case of sequential right ventricular outflow tract obstruction (RVOTO) caused by pulmonary and subpulmonary stenosis, with formation of aortopulmonary collaterals. Case presentation We report a case of a 47 year old Senegalese man who presented with a three year history of shortness of breath on exertion which worsened over the past week. Cardiovascular examination revealed a parasternal heave, a systolic murmur loudest in the pulmonary area and no evidence of fluid overload. A transthoracic echocardiogram revealed leftward septal deviation during systole due to right ventricular pressure overload, severe right ventricular hypertrophy (RV free wall end-diastolic thickness of 8mm) and normal systolic function (FAC 47%), a dilated right atrium and moderate tricuspid regurgitation with estimated maximum pressures of 112mmHg. On continuous-wave doppler of the right ventricular outflow tract (RVOT), there was a late peaking systolic flow with maximum velocity of 3.8m/s. A cardiac computed tomography (CT) and cardiac magnetic resonance imaging (MRI) was performed which showed sequential stenoses of the RVOT; one at subvalvular level by an infundibular muscular ridge with an area of 0.7 cm2 in mid-systole and one at the level of a dome-shaped pulmonary valve with planimetered valve area of 0.5cm2, severe RV hypertrophy and normal RV ejection fraction. Aortopulmonary collaterals from proximal thoracic descending aorta were seen. The main pulmonary artery was shown to be dilated with no evidence of pulmonary artery branch stenosis. See image: In-plane phase contrast velocity flow mapping of the RVOT using a VENC of 80cm/s, showing aliasing at the level of the pulmonary valve (red arrow) and at the level of the infundibulum (white arrow). Conclusion Congenital pulmonary stenosis (PS) occurs in 8% of congenital heart defects. 80% of pulmonary valve stenosis is typically dome-shaped with commissural fusion whilst the remainder is of the dysplastic type. Congenital PS can be associated with RVOTO at the infundibular level secondary to reactive muscular hypertrophy. In our case, there is a discrete, circumferential muscular ridge at the level of the infundibulum resulting in sequential outflow tract obstruction. PS with intact ventricular septum occurs in conjunction with varying degrees of right ventricular hypertrophy and elevated right ventricular systolic pressures. A dilated pulmonary artery is common in dome-shaped subtype of PS. Survival into adulthood of severe PS is primarily dependent on the adequacy of pulmonary blood flow from systemic-to-pulmonary collateral arteries, which serve as an additive, or the only source of blood supply to the pulmonary arterial vasculature. These collaterals are usually seen in association with cyanotic congenital heart disease such as pulmonary atresia and tetralogy of fallot. Abstract P1691 Figure. Sequential RVOTO

P270 Complex cyanotic heart disease in adult patient with down syndrome accompanied by mild pulmonary hypertension

Abstract Funding Acknowledgements No funding Introduction Patients with Down syndrome and complex unrepaired cyanotic heart disease have impaired life expectancy. The clinical status of each patient depends both on the primary lesion type as well as the cummulative haemodynamic burden in the cardiovascular system. Assessment of an adult patient with complex congenital heart disease that has not been surgically repaired presents several challenges since initial haemodynamic parameters and compensatory mechanisms have to be taken into account. Case presentation A 43 year old male patient with known history of Down syndrome and no regular follow up presented to the Outpatient Department of our hospital to undergo pre-operative control for minor soft tissue surgery. The patient exhibited typical morphological features of Down syndrome as well as central cyanosis and digital clubbing. On auscultation a holosystolic type murmur 3/6 on the 3rd left parasternal border was audible. His blood gases revealed hypoxia and mild metabolic acidosis. Electrocardiogram displayed right axis deviation and signs of right ventricular hypertrophy. Transthoracic echocardiography demonstrated a large perimembranous ventricular septal defect (VSD) with normal anatomy and function of the aortic valve and no overiding aorta. Of note was excessive infundibular subpulmonary stenosis which resulted in a functionally double chambered right ventricle. Left ventricle was of normal diamater with good overall systolic function while the right ventricle was non-dilated with significant hypertrophy. Intracavitary gradient was estimated at 60 mmHg. Continuous Doppler showed mild mitral regurgitaion and mild tricuspid regurgitaion with estimated systolic pulmonary pressure of 40 mmHg. Bidirectional flow with low velocities at the level of the defect was recorded. Inferior vena cava was mildly dilated with normal respiratory variation. Discussion/ Conclusion: The natural history of large VSDs results in progressive dilatation of the right ventricle, the left ventricle and the left atrium with gradually increasing pylmonary vascular resistance and development of pulmonary hypertension, followed by right ventricular hypertrophy, flow reversal and Eisenmenger syndrome. However when RVOT obstruction at any level coexists, significant right ventricular hypertrophy develops and the incresed right ventricular systolic pressures acts protectively by minimizing the shunt at the VSD level. Thus a restrictive VSD due to a functionally double chambered right ventricle created a very fragile balance; a cyanotic heart disease with mild bidirectional flow depending on preload and afterload conditions that survived to adulthood. Abstract P270 Figure. Complex unrepaired CHD

P5685Abnormal anatomy of the coronary sinus in congenitally corrected transposition of the great arteries (double discordance): a pitfall for transvenous cardiac resynchronization therapy?

Abstract Background Congenitally corrected transposition of the great arteries (ccTGA) or double discordance is a rare congenital heart defect associating discordant atrioventricular and ventriculoarterial connections. Late prognosis depends on the progressive failure of the systemic right ventricle (RV). A possible cause for systemic RV dysfunction could be ventricular asynchrony. Cardiac resynchronization therapy (CRT) may thus be indicated in some cases. However, the cardiologists may experience some problems with the coronary sinus (CS) when implanting a 3-lead CRT transvenous system in these patients. Aim of the study To evaluate the anatomy of the CS and cardiac veins in specimens with ccTGA, in order to assess the feasibility of transvenous CRT. Material and methods Among the anatomic collection of the French Centre of Reference for complex CHD, 51 heart specimens had ccTGA with 2 ventricles. There were 33 post-natal and 18 fetal hearts. Hearts were reviewed with special attention paid to the course and drainage of the CS and cardiac veins. Segmental anatomy, location of the ventricular septal defect (VSD), status of the pulmonary outflow tract and anomalies of the atrioventricular valves were reviewed. Results Segmental anatomy was {S,L,L} in 46/51 hearts, {S,L,D} in 2 and {I,D,D} in 3. There was a VSD in 40 (outlet in 25, inlet in 11, both in 2, muscular in 2), pulmonary atresia in 13, subpulmonary stenosis in 6, abnormal tricuspid valve in 20/48 including Ebstein anomaly in 6, straddling in 9 (3 had replacement). The CS was always located behind the morphologically left atrium (LA). However, its anatomy was normal, with normal drainage into the morphologically right atrium, in only 25/51 (49% of cases). The CS was of reduced length with normal orifice in 17. Orifice was atretic with normal size CS in 2, and CS was completely absent in 6 with direct drainage of coronary veins into the LA. At least 1 available vein was found in all cases with patent CS orifice. Conclusion CS in ccTGA is always located behind the morphologically LA. However, its anatomy is abnormal in half of cases. The most frequent anomalies are reduced length (33%) and absent CS or atretic orifice (15.5%). The anatomy of CS should therefore be assessed by imaging techniques (multislice CT imaging or CS venography) before considering transvenous CRT in these patients.

Tetralogy of Fallot: morphological variations and implications for surgical repair.

OBJECTIVESTetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in aortic override, coronary arterial patterns and ventricular septal defects in tetralogy of Fallot as observed in a morphological archive, highlighting implications for surgical management.METHODSThe Birmingham Children’s Hospital archive contains 211 hearts with tetralogy of Fallot, of which 164 were analysed [69 (42.1%) unrepaired and 95 (57.9%) operated specimens]. A detailed morphological and geometric analysis was performed using a rigorous 5-layer review process.RESULTSAnomalies were observed in the orifices, origins and course of the coronary arteries: 20 hearts (13.0%) had more than 2 orifices and 3 hearts (1.9%) had a single orifice. In 7 hearts (4.3%), a coronary artery crossed the right ventricular outflow tract. The extent of aortic override ranged from 31.0% to 100% (median of 59.5%). The ventricular septal defect was most often perimembranous (139, 84.8%), but we also found muscular (14, 8.5%), atrioventricular (7, 4.3%) and doubly committed juxta-arterial (2, 1.2%) variants.CONCLUSIONSAnatomical variations are common and can impact surgical management. Anomalous coronary arteries may require a conduit rather than a transannular patch. Variability in aortic override determines the size of patch used to baffle blood to the aorta. The type of ventricular septal defect affects patch closure and the risk of postoperative conduction defects.

An Unusual Complication of Perimembranous Ventricular Septal Defect with Infective Endocarditis: Vegetations Obstructing Right Ventricular Outflow Tract and Pulmonary Valve

Ventricular septal defect (VSD) is usually diagnosed in childhood. In adults, it is less often diagnosed due to spontaneous closure of some VSD's during the early years of childhood. Perimembranous VSD is a defect seen in the upper part of the septum and near the valves occurring in nearly 75% of patients. Although spontaneous closure has been reported in VSD, with about 30%–40% closing before 2 years of age and the remainder of cases closing by about 4 years of age, it is rare for VSD's to close after 8 years of age. Most of the small VSD's will remain asymptomatic, but one of the long-term complications of a small VSD is the development of infective endocarditis (IE). Most of the studies in adults with small VSD's and IE had many complications such as arrhythmias, subaortic and sub-pulmonic stenosis, and exercise intolerance. Here, we report a patient with small perimembranous VSD who was asymptomatic in childhood but suddenly become symptomatic due to development of IE. In this patient, the vegetations were large, multiple chunky linear vegetations partly sealing the VSD defect and also occluding the right ventricular outflow tract and pulmonary valve mimicking severe pulmonary stenosis, which was managed conservatively.

Intracardiac anatomical relationships and potential for streaming in double inlet left ventricles.

The aim of this study was to gain better understanding of the variable anatomical features of double inlet left ventricle hearts without cavopulmonary connection that would potentially facilitate favorable streaming. Thirty-nine post-mortem specimens of double inlet left ventricle without cavopulmonary connection were investigated. The focus was on anatomical characteristics that could influence the flow and separation of deoxygenated and oxygenated blood in the ventricles. Elements of interest were the ventriculoarterial connection, the spatial relationship of the ventricles, the position and size of the great arteries, the ventricular septal defect, the presence of relative outflow tract stenosis and the relationship of the inflow and outflow tracts. The most common anatomy was a discordant ventriculoarterial connection with an anatomically left-sided morphologically right ventricle (n = 12, 31%). When looking at the pulmonary trunk/aorta ratio, 21 (72%) hearts showed no pulmonary stenosis relative to the aorta. The ventricular septal defect created a relative subpulmonary or subaortic stenosis in 13 (41%) cases. Sixteen (41%) hearts had a parallel relationship of the inflow and outflow tracts, facilitating separation of deoxygenated and oxygenated blood streams. On the other end of the spectrum were 10 (25%) hearts with a perpendicular relationship, which might lead to maximum mixing of the blood streams. The relationship of the inflow and outflow tracts as well as the presence of (sub-) pulmonary stenosis might play a crucial role in the distribution of blood in double inlet left ventricle hearts. Additional in vivo studies will be necessary to confirm this postulation.

73 Importance of classification and reassessment in patients with pah-chd: the norpap database

BackgroundPatients with Pulmonary Arterial Hypertension associated with Congenital Heart Disease (PAH-CHD) are subdivided into 4 categories: 1) Eisenmenger Syndrome, 2) PAH with systemic-to-pulmonary shunts, 3) PAH with coincidental defects, 4)...