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BACKGROUND: A 33-year-old adult with complex congenital heart disease and severe cyanosis presented to us with increasing symptoms of hyperviscosity, decreasing exercise tolerance and a thromboembolic complication. INVESTIGATION: Physical examination, laboratory investigations, ECG, transthoracic echocardiogram, cardiopulmonary exercise testing, angiography. DIAGNOSIS: Subvalvular and valvular pulmonary stenosis due to a calcified pulmonary valve. TREATMENT: Amiodarone, angiotensin-converting enzyme inhibitor, beta-blockers, percutaneous pulmonary balloon valvuloplasty, warfarin.

We present the case of a 6-year-old patient with double-orifice mitral valve and severe regurgitation, which was successfully repaired. We demonstrate that mitral valve repair may be attempted even in complex anatomy such as the double-orifice mitral valve, where it can offer excellent results.

A 3-year-old boy underwent evaluation for dextrocardia. Echocardiograms showed features of corrected transposition physiology, a perimembranous ventricular septal defect (VSD) (Fig. 1), and aneurysmal tissue beneath the pulmonary valve that caused severe subpulmonary stenosis (Figs. 2–4). Eighteen months after VSD closure and resection of the aneurysmal tissue, the patient was asymptomatic with only mild residual pulmonary outflow tract obstruction. Fig. 1. Two-dimensional echocardiograms A) in apical 4-chamber view from the right chest and B) in color-flow Doppler mode show a moderate-to-large ventricular septal defect (VSD). Fig. 2. Echocardiograms (subcostal 4-chamber views). A) In the morphologic left ventricle (MLV), aneurysmal tissue (arrow) protrudes into the pulmonary outflow tract. B) Color-flow Doppler mode reveals turbulent flow (arrow) in the pulmonary outflow tract. Fig. 3. A) Continuous-wave Doppler echocardiographic recording across the pulmonary outflow tract shows a peak velocity >5 m/s, suggesting severe obstruction. B) Angiogram (60° left anterior oblique view) from the morphologic left ventricle (MLV) ... Fig. 4. Cineangiographic image from the A) left lateral view reveals the morphologic left ventricle (MLV) and the pulmonary outflow tract obstruction from aneurysmal tissue (arrows). B) The 60° left anterior oblique view reveals a severely narrowed right ...

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (

Transthoracic echocardiography is the first-line modality for cardiovascular imaging in adults with congenital heart disease (ACHD). The windows of access that are possible with transthoracic echocardiography are, however, rarely adequate for all regions of interest. The choice of further imaging depends on the clinical questions that remain to be addressed. The strengths of MRI include comprehensive access and coverage, providing imaging of all parts of the right ventricle, the pulmonary arteries, pulmonary veins and aorta. Cine images and velocity maps are acquired in specifically aligned planes, with stacks of cines or dynamic contrast angiography providing more comprehensive coverage. Tissues can be characterised if necessary, and MRI provides relatively accurate measurements of biventricular function and volume flow. These parameters are important in the assessment and follow-up of adults after repairs for tetralogy of Fallot or transposition of the great arteries and after Fontan operations. The superior spatial resolution and rapid acquisition of CT are invaluable in selected situations, including the visualisation of anomalous coronary or aortopulmonary collateral arteries, the assessment of luminal patency after stenting and imaging in patients with pacemakers. Ionising radiation is, however, a concern in younger patients who may need repeated investigation. Adults with relatively complex conditions should ideally be imaged in a specialist ACHD centre, where dedicated echocardiographic and cardiovascular MRI services are a necessary facility. General radiologists should be aware of the nature and pathophysiology of congenital heart disease, and should be alert for previously undiagnosed cases presenting in adulthood, including cases of atrial septal defect, aortic coarctation, patent ductus arteriosus, double-chambered right ventricle and congenitally corrected transposition.

BACKGROUND A discrete subaortic membrane cannot only cause left ventricular outflow tract obstruction, but can grow onto the aortic valve leaflets. The late finding of this encroachment is aortic valve insufficiency or stenosis. Echocardiography is used to follow the progression of outflow tract obstruction, but its ability to show subaortic membrane encroachment onto the aortic valve is unclear. The purpose of this study is to determine the sensitivity and specificity of echocardiography for diagnosing whether a discrete subaortic membrane involves the aortic valve. METHODS A pre-operative determination of aortic valve involvement by a discrete subaortic membrane was obtained by review of the official pre-operative echocardiogram reading and a retrospective blinded review of the pre-operative echocardiogram by an independent echocardiographer. These findings were compared to the intra-operative findings. RESULTS A total of 48 consecutive patients underwent primary resection for isolated discrete subaortic membrane between October, 1995 and May, 2006. The pre-operative and blinded readings both predicted a statistically lower rate of aortic valve involvement - 35% in 11 of 31 patients and 31% in 10 of 31 patients, respectively - than found at surgery - 65% in 31 of 48 patients. The sensitivity and specificity of pre-operative echocardiography to diagnose aortic valve involvement is 35% and 76%. Overall survival was 100%. There were no strokes, re-operations for bleeding or wound infections, or need for a pacemaker. CONCLUSION Echocardiography is not sensitive in assessing whether a discrete subaortic membrane involves the aortic valve. Since the morbidity and mortality for discrete subaortic membrane resection is negligible, resection may be indicated at the time of diagnosis to minimise aortic valve impairment.

Ventricular septal defect associated with infundibular pulmonary stenosis is a relatively uncommon congenital cardiac defect. We report the first case of a patient with perimembranous small ventricular septal defect and infundibular stenosis suffered from pulmonary valve endocarditis and septic pulmonary embolism.

Objectives To evaluate the natural history of primary pulmonic infundibular stenosis in cats and the effects of balloon valvuloplasty. Background Primary pulmonic infundibular stenosis is an uncommon congenital defect in cats. The natural history of the disease has not been described. Information regarding balloon valvuloplasty in the cat is limited. Animals Records between January 1, 1999 and December 31, 2005 were reviewed and cats with a confirmed echocardiographic diagnosis of primary pulmonic infundibular stenosis, a complete medical history, and no evidence of significant systemic disease were identified. Methods Echocardiographic, electrocardiographic, and radiographic findings are described. The natural history of those with severe disease was compared to those with mild to moderate disease. Balloon valvuloplasty was performed in six of the cats. The technique used is described. Results A stenotic gradient ≥70 mmHg and a right ventricular outflow tract (measured at the level of the stenosis) to pulmonary valve annulus ratio of ≤0.25 were consistent with clinical and echocardiographic severe disease. Cats with severe disease had a very guarded prognosis with a high incidence of congestive heart failure (CHF). Balloon valvuloplasty was successfully performed in 4 of the cats and appeared to improve prognosis, especially if performed prior to the development of CHF. Concurrent hypertrophic cardiomyopathy complicated the outcome in some cats. Conclusions Severe primary pulmonic infundibular stenosis carries a very guarded prognosis in the cat. Balloon valvuloplasty should be considered in the presence of severe disease and should be performed prior to the development of CHF if possible. The presence of concurrent hypertrophic cardiomyopathy may complicate the prognosis.

This case demonstrates the development of secondary infundibular stenosis in a 10-year-old male child with documented large non-restrictive perimembranous ventricular septal defect in infancy – the classical Gasul phenomenon.

Isolated subvalvular pulmonary stenosis is a rare condition and its morphological evaluation is obscure. Whole heart magnetic resonance imaging (MRI) is a new, totally non-invasive technique which allows three-dimensional comprehension of the cardiac structure. We describe a patient with isolated subvalvular pulmonary stenosis, in whom whole heart MRI was useful to detect and evaluate the right ventricular outflow obstruction.

An increasing number of patients with tetralogy of Fallot (TOF) are reaching older age. We encountered a 75-year-old woman with uncorrected TOF and concomitant severe coronary artery disease (CAD) with congestive heart failure. Her CAD risk factor was hyperlipidemia, which had been untreated. Successful percutaneous coronary interventions have improved her clinical condition and provided long-term survival. Although CAD is considered to be a rare complication in adults with TOF, both strict modification of CAD risk factors and early detection of CAD would be also required in this population, given the residual TOF lesions relating to acute exacerbation of clinical presentation.

OBJECTIVES We sought to highlight the clinical, morphologic, and pathogenetic features in patients with a cleft mitral valve (MV). BACKGROUND Few studies have addressed the morphologic features of cleft MV and the outcome of these patients. The pathogenetic features, including the developmental relation to an atrioventricular (AV) septal defect, remain unclear. METHODS We reviewed the patients with cleft MV that were diagnosed by echocardiography since 1980. Patients with an AV canal, ventriculo-arterial discordance, and hypoplastic ventricles were excluded. RESULTS Twenty-two patients were identified at a median age of 0.5 years (range 0 to 10.6). In three patients, no chordal attachments of the cleft to the ventricular septum were seen. Ten patients had significant mitral regurgitation (MR), and three had subaortic obstruction by the cleft. Associated cardiac lesions and extracardiac features were present in 13 and 10 patients, respectively. During the median follow-up period of 1.5 years (range 0 to 11.8), two patients died of extracardiac causes, and one neonate died of severe subaortic obstruction. Surgical repair was performed in 10 patients at a median age of 5.2 years (range 1.3 to 10.6). Multivariate analysis showed no predictors for MV surgery. One patient was re-operated for mitral stenosis associated with aortic valve stenosis. Follow-up echocardiography demonstrated moderate MR in two unoperated patients and moderate MV stenosis in two operated patients. CONCLUSIONS A cleft of the MV comprises a wide spectrum. Important morphologic differences exist with an AV septal defect, although the two lesions may be pathogenetically related. Surgical repair always seems possible. Long-term echocardiographic follow-up is warranted.

An arterial switch is the corrective procedure of choice for d-transposition of the great arteries but may be associated with increased morbidity and mortality when performed in low-birth-weight infants. Conversely, delaying surgery often leads to left ventricular "deconditioning" as pulmonary arteriolar resistance decreases. We present an infant with a birth weight of 940 g with d-transposition of the great arteries with an intact ventricular septum whose bilateral pulmonary artery branch stenosis allowed for maintenance of near systemic left ventricular pressure, thereby protecting against deconditioning. This case also represents the smallest reported patient to undergo a successful balloon atrial septostomy.

Though acute and follow-up benefits of pulmonary valve balloon dilatation (PVBD) for pulmonic valve stenosis are well known, the late course of residual gradients at individual valvular and infundibular levels is not well described. Furthermore, the factors influencing this late course have not been studied.We assessed the transpulmonary gradients by echo-Doppler in 96 patients (61 male, mean age 10.7 years) at a mean follow-up interval of 58.8+/-32.1 months (minimum 2 years) following PVBD. The patients were divided into three groups based on the residual gradients at valvular and infundibular levels immediately following PVBD as assessed by pull-back of an end-hole catheter across the right ventricular outflow tract: Group A with minor pressure gradients at both valvular and infundibular levels of less than 30 mmHg (n=60, 62.5%), Group B with predominantly infundibular gradients of more than 30 mmHg with a valvular gradient of less than 30 mmHg (n=27, 28.1%), and Group C with a residual valvular gradient of greater than 30 mmHg irrespective of the infundibular gradient (n=9, 9.4%). Demographic characteristics, hemodynamic parameters and procedural variables were correlated with the change in gradient at follow-up (late fall) and with long-term results.The mean follow-up trans-pulmonary gradient was 20+/-14 mmHg which was significantly lower than that immediately post-PVBD (43+/-32 mmHg), P0.001. The late fall (mean 24+/-29, range -55 to 110 mmHg) varied widely depending upon the acute result group: patients in Groups A and B showed significant late fall of 9+/-12 mmHg (P0.05 for follow-up gradient compared to that following PVBD) and 58+/-31 mmHg (P0.0001), respectively, while patients in Group C showed an insignificant late fall of 14+/-37 mmHg (P=0.21). In particular, each one of the patients in Group B showed decrease in trans-pulmonary gradients. On multivariate analysis, the extent of infundibular gradient emerged as the most important predictor of late fall (coefficient of determination 75%, P0.0001). Patients who underwent PVBD at less than 2 years of age had a significantly greater late fall (41+/-33 mmHg) as compared to older patients (22+/-25 mmHg), P0.05. A sub-optimal long-term result (transpulmonary gradient25 mmHg, n=24) was significantly related to older age (P0.001), dysplastic valve morphology (P=0.002), greater baseline trans-pulmonary gradients (P0.001) and higher post-PVBD gradients (P=0.04).The long-term course of patients following PVBD depends upon the site and magnitude of the residual gradients. Even high residual infundibular gradients show marked reduction at follow-up, especially in infancy.

The management of adult congenital heart disease is challenging and poses specific problems. We report a patient with ventricular septal defect and pulmonary stenosis who underwent successful repair and coronary artery bypass grafting at the age of 76 years.

We analyzed shortening patterns of the infundibulum in 11 patients with valvular pulmonary stenosis (PS) before and immediately after balloon valvuloplasty and at follow-up. The control group consisted of 32 patients with Kawasaki disease. The valvuloplasty was performed at the age of 5.8 +/- 2.9 (mean +/- SD) years and a satisfactory relief of obstruction was achieved in each. The follow-up study was done at 1.1 +/- 0.2 years after the valvuloplasty. With the use of lateral projection of right ventriculograms, transverse diameters of the midinfundibulum were measured over one cardiac cycle from initiation of the pulmonary valve opening. Indices demonstrating shortening patterns of the infundibulum were as follows: time to the beginning of shortening (TBS), time to the shortest diameter (TSD), and shortening fraction (SF). TBS were prolonged before the valvuloplasty, whereas it was comparable with that in the control group, immediately after the procedure and at follow-up. TSD was increased before and immediately after the valvuloplasty, but it was normalized at follow-up. SF was increased at every observation. Thus, shortening patterns of the infundibulum in PS were characterized by increasing TBS, TSD, and SF. At short-term follow-up following the valvuloplasty, TBS and TSD were normalized, which could be a result of a successful relief of pulmonary obstruction. In contrast, SF remained elevated, which indicates that the musculature of the infundibulum remains hypertrophic up to 1 year after the valvuloplasty.

We present a case of a 69 year-old woman complaining of palpitations and worsening heart failure, after the 2nd cycle of fludarabine due to Hodgkin's lymphoma. Echocardiography revealed abnormalities typical for corrected transposition of the great arteries (CTGA), which were confirmed in multislice computed tomography (MSCT), as well as subvalvular pulmonary stenosis. There is the case of one of the oldest patients with CTGA and subvalvular pulmonary stenosis. MSCT seems to be a useful and complementary method in diagnosis such diseases.

Received 20 August 2014; revision accepted for publication 25 November 2014. We describe the case of a 17-year-old girl with a recent diagnosis of hypertrophic cardiomyopathy. Her main symptom was post-exercise dizziness without loss of consciousness. Two-dimensional transthoracic echocardiography revealed marked hypertrophy of the basal segment of the interventricular septum (27 mm) (figure 1, panel A) with no subaortic gradient at rest and during a Valsalva manoeuvre performed in the supine position (figure 1, panel B). The additional investigations did not reveal sustained or non-sustained ventricular tachycardia on 48-hour Holter monitoring nor hypotension during bicycle exercise testing. The patient underwent exercise echocardiography in a semi-supine position on a tilting exercise table. Echocardiographic imaging was continuous during exercise and recovery and did not reveal left ventricular outflow tract obstruction (LVOTO) (figure 1, Hypertrophic cardiomyopathy: obstruction fl ushed out with exercise echocardiography

Ebstein's anomaly comprises a wide spectrum of congenital cardiac abnormalities involving the tricuspid valve (TV) apparatus and the architecture of the morphological right ventricle (RV). We report a case of Ebstein's anomaly in a 3-year-old boy having a unique association with severe subpulmonic obstruction caused by tethered and dysplastic anterior tricuspid leaflet (ATL), and a large ventricular septal defect. In view of poorly functioning RV, the baby was planned for a bidirectional Glenn shunt with subsequent Fontan completion.

Background Certain conditions, including discrete subaortic stenosis and right ventricular outflow tract obstruction are well-known associations with ventricular septal defect. However, the association of discrete type subpulmonary and subaortic stenosis with ventricular septal defect has not been described to date. Patients In this report we present our experience in nine patients with doubly committed subarterial ventricular septal defect in whom associated discrete subpulmonary and subaortic stenosis were diagnosed by two-dimensional echocardiography. The mean age of the patients was 5·7 years, and eight of them were male. Two patients had additional hypertrophied anomalous muscle bundles with pressure gradients across the right ventricular outflow tract. Aortic valve prolapse was detected in two patients with one of them having mild aortic regurgitation. Intervention Cardiac catheterization was performed in four patients, and three underwent surgery. Doubly committed subarterial ventricular septal defect was closed, and subpulmonary and subaortic ridges were resected in each patient. Histological findings were similar for both ridges, which were composed of thin, short and irregularly arranged elastic fibres covered by endothelial cells. Results The finding of fibrous ridge on both sides of the doubly committed ventricular septal defect, where the turbulence is likely to be maximum, and the histopathological similarities of subpulmonary and subaortic ridges, may suggest a common mechanism for the development of fibrous ridges. Conclusion Serial echocardiographic assessment of patients with doubly committed ventricular septal defect is recommended.

This study addresses the effects of early banding of the pulmonary trunk and subsequent management of sub-aortic obstruction on the attainment of acceptable pre Fontan hemodynamics in patients with a single left ventricle and aorta arising from an outflow chamber. We report our experience with 26 patients seen at our institution between January 1984 and December 1994 with a diagnosis of double-inlet left ventricle or tricuspid atresia and transposed great arteries, who were initially managed with pulmonary artery banding in the first 6 months of life. Pulmonary artery band placement was performed at an age of 2.1 ± 1.8 months (mean ± SD). Associated aortic arch abnormalities were present in 8 patients (31%). There were 19 patients (73%) who underwent treatment with a Damus-Kaye-Stansel procedure or ventricular septal defect (VSD) enlargement for a significant subaortic gradient or morphologically small VSD, alone or in conjunction with a Glenn or Fontan procedure. Eighteen of 26 patients (69%) underwent cardiac catheterization to assess their candidacy for the Fontan operation. Of this group, 16 were classified as low to moderate risk and 2 as high-risk Fontan candidates, based on hemodynamic criteria. The cumulative mortality for the entire cohort was 19%. Our results suggest that this high-risk group of patients can undergo effective pulmonary artery banding as an initial palliative step, with subsequent intervention for subaortic obstruction when it is documented or highly suspected, and that acceptable pre-Fontan hemodynamic parameters can be achieved.

Background: One-stage repair of interrupted aortic arch, ventricular septal defect, and severe subaortic stenosis represents a surgical challenge. Techniques that use extracardiac conduits to bypass the subaortic area or involve transaortic or transatrial resection of the conal septum have shown limitations and have failed to reduce the high mortality rate associated with subaortic obstruction.Methods and results: A new operative approach was used in nine neonates (2.1 to 3.9 kg) who underwent one-stage repair of interrupted aortic arch (type B, eight patients; type C, one patient), ventricular septal defect, and severe subaortic stenosis. All patients had severe subaortic stenosis according to preoperative echocardiography (mean ratio of subaortic to descending aortic diameter, 0.63 ± 0.08). With a transpulmonary (seven patients) or transatrial (two patients) approach and without resection of the conal septum, the ventricular septal patch was placed on the left side of the septum to deflect the conal septum anteriorly and away from the subaortic area. There were no early or late deaths. Median intensive care unit and hospital stays were 17 days (6 to 47 days) and 21 days (10 to 55 days), respectively. On follow-up echocardiography (1 to 29 months, median 12 months), no patients had significant residual subaortic obstruction and one patient had mild residual arch obstruction (20 mm Hg). Growth of the subaortic region was demonstrated in all patients (mean ratio of subaortic to descending aortic diameter, 1.20 ± 0.10

We report a case of a 13-year old girl with pectus excavatum who had a Nuss procedure and two years later a new cardiac murmur appeared which on investigation was diagnosed as supravalvular pulmonary artery stenosis. Following removal of the Nuss bar the stenosis resolved.

Three eight-week-old golden retriever puppy littermates were evaluated because of left basilar systolic murmurs and were diagnosed with primary infundibular stenosis. Pedigree analysis in this line was also performed to identify a mode of inheritance. All dogs were asymptomatic at the time of diagnosis; two of the three had congenital lesions in addition to primary infundibular stenosis. Two additional affected dogs were identified in the line, and pedigree analysis suggested an autosomal recessive mode of inheritance. Another, unrelated golden retriever was also identified with isolated infundibular stenosis in the record database. Primary infundibular stenosis should be considered in the differential diagnoses for golden retriever dogs with a left basilar systolic murmur, and is often associated with complex congenital cardiac disease. Primary infundibular stenosis may worsen in severity with time, and in this line of dogs an autosomal recessive pattern of inheritance is likely.

[Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4][![Graphic][5] ][5] A 10-year-old female patient presented with dyspnea during play with peers. She had been diagnosed with a large perimembranous ventricular septal defect neonatally but had since been lost to follow-up. On

Clinical, echocardiographic, hemodynamic, and angiographic findings were reviewed in 16 consecutive patients with tricuspid atresia (TA) and normally related great arteries, seen at the University of Florida between 1984 and 1990, to determine when and in which patients hypercyanotic spells (HS), unrelated to ductal constriction, occurred. Such spells developed in six patients (38%) and were life-threatening and refractory to medical management in three patients. Most HS (five of six) occurred in the first 2 months of life. Angiography done at the time of diagnosis revealed similar morphological features in all patients subsequently experiencing HS; infundibular stenosis due to anterior malalignment of the conal septum. These findings suggest a predisposition for development of early HS in TA patients with apparent infundibular stenosis due to anterior malalignment of the conal septum. Further study and a heightened clinical awareness are needed in such patients.

Objectives. We sought to study the range of conal morphology in transposition of the great arteries with ventricular septal defect and their embryologic and surgical implications. Background. Conal anatomy in transposition of the great arteries aad ventricular septal defect is variable and might affect surgical repair. Methods. Conal anatomy was explored using two-dimensional echocardiography in 119 patients with transposition of the great arteries and a large ventricular septal defect who presented between 1984 and 1991. The influence of conal anatomy on surgical technique was determined by review of the operative reports. Specimens of transposition of the great arteries with unusual conal anatomy were selected from the Cardiac Registry for comparison with the echocardiograms. Results. One hundred five patients (88.2%) had subaortic conus only with no subpulmonary conns (Group 1). Subarterial conus was present bilaterally in eight patients (6.7%) (Group 2). Four patients (3.4%) had only subpulmonary conus with no (or minimal) subaortic conus (Group 3). Among these four patients, the aorta was posterior to the pulmonary artery in one patient, side by side relative to the pulmonary artery in two patients and slightly anterior in the fourth patient. Subarterial conus was absent bilaterally in two patients (1.7%) (Group 4); the aorta was slightly posterior in one and side by side with the pulmonary artery in the other. Conclusions. This variability of conal anatomy in transposition of the great arteries with ventricular septal defect implies four mechanisms by which transposition can occur. The conal anatomy appeared to affect surgical repair in Groups 1 and 2 insofar as it influenced ventricular outflow tract obstruction. In Groups 3 and 4, an arterial switch operation was performed in four of the six patients. The posterior location of the aorta obviated the need for the Lecompte maneuver in two of these four patients. In the remaining two cases in Groups 3 and 4, the condition was repaired by directing the left ventricular outflow across the ventricular septal defect to the aorta using a patch, with or without placement of a conduit from the right ventricle to the pulmonary artery.

Whole-heart magnetic resonance imaging (MRI) was performed in a 59-year-old man who was referred to the department of cardiology because of effort dyspnea. Echocardiography revealed asymmetric hypertrophy of the interventricular septum (Figure 1; upper left panel). There was an obstruction of the right ventricular outflow tract during systole (Figure 1; arrow in upper right panel). Continuous wave Doppler (Figure 1; lower panel) revealed a maximum velocity of 3.0 m/s, which corresponded to a pressure gradient of 36 mmHg within the right ventricular outflow tract. A whole-heart MRI from the short-axis view showed asymmetric hypertrophy of the interventricular septum, which protruded into the right ventricular outflow tract (Figure 2; arrow). Cine MRI also showed asymmetric hypertrophy of the inter-ventricular septum, which obstructed the right ventricular outflow tract during systole (Figure 3; arrows). Figure 1 Figure 2 Figure 3 Isolated subvalvular pulmonary stenosis (ISPS) – subvalvular pulmonary stenosis without a ventricular septal defect – is a rare disorder that has been shown to be associated with right ventricular outflow obstruction in patients with hypertrophic cardiomyopathy (1,2). It is usually accompanied by left ventricular outflow obstruction, but isolated right ventricular obstruction occurs infrequently. In contrast to conventional MRI, which requires prospective projection settings for constructing the multiple cross-sectional images, whole-heart MRI can retrospectively depict arbitrary cross-sectional images from the previously acquired three-dimensional data. In addition, whole-heart MRI allows for evaluation of the anomalous coronary artery that often accompanies ISPS (3). Thus, whole-heart MRI has the potential to become a routine diagnostic modality in patients with suspected ISPS.

Objective: Anatomically corrected malposition of great arteries (ACMGA) is associated with the leftward deviation of the proximal right coronary artery (RCA) away from the right atrioventricular (AV) groove. This anatomic feature allows a transannular subpulmonary patch plasty along the right AV groove between the RCA and the tricuspid annulus for relief of subpulmonary stenosis. This technique is also applicable to similar malformations that belong to the same spectrum. The long-term results of this original method and its modification were reviewed. Methods:Thissurgicaltechniquewith a monocuspidtransannularpatch for subpulmonary stenosiswas appliedin13 patients(seven patientswith ACMGA, four patients with transposition of the great arteries after arterial switch operation (Shaher type 4 or 9) who underwent the original Konno or subaortic resection simultaneously, and two patients with discordant AV connection who have undergone conventional left ventricle to pulmonary artery repair or ventricular septation and pacemaker implantation previously). The mean age at operation was 15 years. Results: Postoperative catheterisation revealed adequate relief of pulmonary stenosis, with a pressure gradient of 5.6 4.5 mmHg and with normalised right ventricular pressure (35 12 mmHg). There was no late death or re-operation during a postoperative follow-up period of 90 58 months. All patients were in normal sinus rhythm, postoperatively, except for 3 patients (one AV block due to subaortic resection, and two pacemaker rhythms implanted previously). Conclusions: The present report describes the excellent long-term results of the AV groove patch plasty for subpulmonary stenosis in ACMGA and similar malformations by using morphological advantages. This technique provides a promising alternative to the Rastelli-type conduit repair. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

A 35-year-old man with a history of syncope and echocardiographic diagnosis of right ventricular outflow tract obstruction and ventricular septum defect was submitted to complete evaluation with an Aquilion Toshiba 64-multislice CT in order to confirm diagnosis and to complete information prior to surgery.

Left ventricular pacing via the coronary sinus is being increasingly used. There is little data to guide possible lead extractions that might be required in the future. Significant adhesions to the coronary veins were found 12 years after placing a pacing lead in the posterolateral coronary vein in a man with double inlet left ventricle and severe subpulmonary stenosis who had undergone a Fontan operation. The appearances suggest that percutaneous extraction from the proximal coronary sinus may be feasible but that difficulty may be encountered if the lead tip is placed into the distal coronary veins.

In order to clarify cross-sectional morphology of tetralogy of Fallot in the fetus, 202 rat fetuses with tetralogy of Fallot induced by maternal administration of bis-diamine were studied using cesarean section, the whole body freezing method and by photographing cross sections of the fetal heart on a freezing microtome. Some characteristic features such as large subaortic ventricular septal defects, aortic overriding over the ventricular septum, aortomitral fibrous continuity, and narrow infundibulum and pulmonary valve were found. Moreover, three subtypes were noticed. In classic tetralogy of Fallot with stenotic infundibulum, the size of the pulmonary arteries was smaller in proportion to the infundibular stenosis. In the second type with absent infundibular septum and severe valvular pulmonary stenosis, the pulmonary arteries were most hypoplastic. In the third type with absent pulmonary valve, the pulmonary arteries were dilated forming aneurysms. In the last type only, ventricles were dilated and grossly hypertrophic.

Pulmonary stenosis (PS) can be seen from the right ventricular outflow tract to the peripheral pulmonary arteries. Most frequently, the obstruction occurs at the level of the pulmonary valve; however, it occurs less frequently at the infindibular level within the trabecular component of the right ventricle or within the pulmonary arterial pathways. Lesions at any of these levels can occur as part of more congenital cardiac malformations such as tetralogy of Fallot, complete transposition of great arteries, or atrial septal defect. Isolated supravalvular pulmonary stenosis (iSPS) is less common than other types of PS. In this study, we present our experience with 4 patients who underwent cardiopulmonary bypass operation for iSPS. In one patient, the circular stenotic area was noted on the touch point of the pulmonary valve. Right ventricular pressures ranged from 70 to 90 mmHg, and the pulmonary artery mean pressures ranged from 14 to 17 mmHg. In all patients, the left ventricular and aortic systolic, diastolic, and mean pressures were moderately increased. Pulmonary artery stenosis was treated successfully using a pericardial or Dacron patch on cardiopulmonary bypass. Various techniques such as balloon dilation have been proposed to deal with this problem, but these may often be unsuccessful because of the elasticity and recoil of the pulmonary artery constrictive ring. Even though endovascular stenting and/or balloon angioplasty have been recently proposed as an initial treatment strategy, they may be associated with some severe complications including pulmonary artery thrombosis or stent migration. Our study, even though it consists of a limited number of cases, suggests that open heart surgery using an oval-shaped patch may be a used as the other main choice for the treatment of iSPS.

I infundibular pulmonic stenosis is an uncommon cause of right ventricular (RV) outflow tract obstruction.1–3 It is a discrete subvalvular fibromuscular obstruction.4 Surgical resection of the fibromuscular hypertrophy remains the treatment of choice when there is significant hemodynamic compromise. The optimal time of surgery should be before the development of RV failure. However, surgical correction requires extracoporeal circulation and carries a risk of mortality of about 5% to 10%.2,3 Percutaneous balloon valvuloplasty is also an attractive technique, but it is only partially effective in the treatment of infundibular stenosis.5 Recently, the transcoronary alcohol ablation technique has been reported to be useful in the treatment of idiopathic hypertrophic cardiomyopathy.6,7 In this report, we describe a patient with idiopathic infundibular pulmonic stenosis in whom nonsurgical ablation of the infundibular hypertrophy was successful. • • • A 47-year-old man was admitted to the hospital with a 3-year history of progressive dyspnea. He was well until 3 years earlier, when he began to experience dyspnea on exertion. He consulted his primary physician and was treated with diuretics. His symptoms continued. Two months before admission, dyspnea resulted in marked limitation of ordinary physical activity (New York Heart Association functional class III). He had a 5/6 systolic ejection murmur at the left sternal border. The electrocardiogram showed RV hypertrophy. The echocardiogram revealed a pressure gradient of 100 mm Hg across the RV outflow tract, normal pulmonic valve, no evidence of left to right shunt, normal left ventricular wall thickness, and normal left ventricular function. Right cardiac catheterization showed isolated infundibular stenosis of the RV outflow tract with a pressure gradient of 90 mm Hg. RV angiography confirmed the diagnosis of isolated infundibular pulmonic stenosis with normal RV systolic function (Figure 1). Surgical resection was recommended, but he refused it. Nonsurgical treatment was considered for symptomatic relief. Based on the nonsurgical ablation technique of hypertrophic cardiomyopathy, informed consent for alcohol ablation of the infundibular hypertrophy was obtained from the patient. A right coronary angiogram showed an abnormally well-developed conus branch (Figure 2). It mainly supplied the hypertrophied myocardium of the RV outflow tract, which was delineated by myocardial contrast echocardiography. The conus branch was selectively catheterized with a 2-mm angioplasty balloon positioned at the proximal portion of the conus branch. Pressure gradient across the RV outflow tract was decreased from 90 to 70 mm Hg during balloon inflation. The effects of balloon occlusion were reversed immediately after balloon deflation. After intravenous administration of 4-mg morphine, 5-ml absolute alcohol was slowly injected through the inflated balloon into the vessel and left for 3 minutes before the balloon was deflated. The RV outflow tract pressure gradient immediately decreased from 90 to 60 mm Hg after alcohol injection (Figure 3). The patient complained of mild chest pain during the first day after alcohol ablation. The electrocardiogram showed ST-segment elevation at precordial leads V1 to V3, which subsequently returned to baseline with poor progression. Atrial flutter was transiently developed during the first day after alcohol ablation. Creatine phosphokinase activity rose to a maximum of 2500 IU/L 8 hours after the procedure. Echocardiography after the procedure showed normal left ventricle wall motion and akinesia of the anterior wall of the RV outflow tract. The patient was discharged without complications 10 days after alcohol ablation. Right cardiac catheterization 1 month after alcohol ablation showed a pressure gradient of 30 mm Hg across the RV outflow tract (Figure 3). RV angiography also showed normal RV contractility, except for akinesia of the anterior wall of the RV outflow tract. The diameter of the most stenotic portion increased from 3 to 7 mm 1 month after alcohol ablation. RV function evaluated by multiple gated image acquisition analysis scan did not significantly change 1 month after alcohol ablation (ejection fraction: 60% vs 65%, before and after ablation, respectively). The patient had an uneventful recovery and his symptoms disappeared. At 3-month follow-up, the patient said that he could perform to completion any physical activity without symptoms. • • • Infundibular hypertrophy may develop in a variety of conditions, such as severe pulmonic stenosis, and aneurysm of the From the Department of Medicine, University of Ulsan, Asan Medical Center, Seoul, Korea. Dr. Park’s address is: Department of Medicine, Asan Medical Center, University of Ulsan, Kang-Dong P.O. Box 145, Seoul, 134-600, Korea. Manuscript received May 6, 1997; revised manuscript received and accepted August 15, 1997.

48-year-old man was referred for radiofrequency (RF) blation of atypical atrial flutter. He had undergone repair of ventricular septal defect and resection of a right ventriclar infundibular stenosis at age 36 years. At electrophysiologic study, slow atrial flutter with a ycle length of 300 ms was present. Activation mapping f the enlarged right atrium was performed, and a line of ouble potentials was identified as the old atriotomy scar Figure 1, blue dots). However, the tachycardia mechaism remained unclear. Was the mechanism a reentry ounterclockwise around the tricuspid annulus or a reenry clockwise around the atriotomy scar? What should be one next?

Objective: In anatomically corrected malposition of the great arteries, dextroposition of the posterior pulmonary artery and levoposition of the anterior aorta are associated with the leftward deviation of the proximal portion of the right coronary artery away from the right atrioventricular groove. This anatomic feature allows a transannular subpulmonary patch plasty of the right ventricular outflow tract along the right atrioventricular groove between the right coronary artery and the tricuspid anterior anulus (ie, atrioventricular groove patch plasty) for relief of subpulmonary stenosis without jeopardizing the right coronary artery. Methods: This report describes the midterm results of a new surgical technique, atrioventricular groove patch plasty with a monocuspid transannular patch for subpulmonary stenosis, in 3 patients with anatomically corrected malposition of the great arteries, along with a concomitant closure of ventricular septal defects. Results: Postoperative catheterization revealed adequate relief of pulmonary stenosis, with a pressure gradient of 8.0 ± 3.5 mm Hg and with normalized right ventricular pressure (33 ± 10 mm Hg), contributing to excellent midterm results with no late death and reoperation during a postoperative follow-up period of 70 ± 47 months. Conclusion: This technique provides a promising alternative to Rastelli-type conduit repair for subpulmonary stenosis in anatomically corrected malposition of the great arteries. J Thorac Cardiovasc Surg 2001;122:872-8

A neonate with L-transposition of the great arteries with ventricular septal defect underwent complete repair using fresh autologous pericardium to close the ventricular septal defect as well as to reconstruct the neo pulmonary artery sinuses. Four months later, the child came back with right ventricular inflow obstruction related to aneurysmal pericardial patch, severe tricuspid regurgitation, and severe supra-valvular pulmonic stenosis. At reoperation, there was a redundant, aneurysmal pericardial patch densely adherent to the septal and posterior leaflets of the tricuspid valve, which was damaged. The pericardial patch was replaced, the pulmonary artery enlarged, and tricuspid valve repaired. Postoperative course was uneventful, but residual moderate tricuspid regurgitation required intensive medical treatment.

It has been shown that dualchamber pacing of the right heart may decrease the pressure gradient in hypertrophic obstructive cardiomyopathy of the left ventricle. However, to our knowledge, no data are available on the effects of left ventricular pacing in patients with dynamic intracavitary obstruction of the right ventricle due to pulmonary infundibular stenosis. This report evaluates acute effects of temporary DDD pacing (right atrium sensed and left ventricle paced) in 3 symptomatic patients with fibromuscular type of pulmonary infundibular stenosis. Informed consent was obtained from each patient before the study.

Background. Until recently closure of the pulmonary valve during staged Fontan-type palliation in the setting of double-inlet left ventricle with an unrestrictive or adequately enlarged ventricular septal defect and transposition of the great arteries with the aorta on a left-sided outflow chamber was regarded as an appropriate part of surgical treatment. Lately, however, an increased incidence of subsequent subaortic obstruction has been described in this regard. Methods. Allograft root placement on the previously closed pulmonary orifice in combination with a modified Damus-Kaye-Stansel procedure is described to create an unobstructed outflow from the main ventricle to the systemic circulation. This procedure was done in 3 patients. One root placement was combined with the construction of the bidirectional superior cavopulmonary connection, one was done as an intermediate step before completion of the cavopulmonary connection, and one was combined with completion of total cavopulmonary connection. Results. Immediate relief of the subaortic obstruction was achieved in all 3 patients. Ventricular hypertrophy, echocardiographically assessed by diastolic posterior wall thickness, regressed to normal in all 3 within 6 to 12 months. Conclusions. Allograft root placement on the reopened pulmonary orifice in double-inlet left ventricle with a ventricular septal defect and transposition of the great arteries appears technically feasible and functionally adequate on short-term follow-up. This procedure should result in regression of ventricular hypertrophy to allow eligibility for a Fontan-type palliation again. To what extent possible failure of the allograft increases the risk of an adverse outcome of this palliation may be a matter of concern.

We present the case of a male patient with subvalvular aortic stenosis, subvalvular pulmonary stenosis and membranous ventricular septal defect. The patient was admitted to our clinic due to increasing exertional dyspnoea after suffering from mild exertional dyspnoea and easy fatigue for many years. A balloon valvuloplasty of the stenosed pulmonary valve was successfully performed 15 years ago. On transthoracic echocardiographic examination, mildly hypertrophied left ventricle (LV) with preserved systolic function was found. Aortic valve was literally degenerated with moderate aortic regurgitation. Below the aortic valve, a membrane partially obstructing the LV outflow tract was found (Fig. 1A–C). In the membranous part of the interventricular septum, a left to right shunt was noted with maximum flow velocity of 5 m/s (Fig. 1D–E). The right ventricle was mildly hypertrophied; examination of the pulmonary valve revealed slightly degenerated leaflets with good separation and mild regurgitation although the measured peak pressure gradient was increased to 40 mm Hg. In the short axis view, a membrane in the right ventricular outflow tract below the pulmonary valve was found (Fig. 1D). The patient was referred for cardiac surgery. The diagnosis was confirmed at surgery. A resection of the subaortic membrane was performed and the aortic valve was replaced by a mechanical prosthesis. The ventricular septal defect was closed. The subpulmonary membrane was resected and epicardial radiofrequency ablation was performed. The postoperative control echocardiography showed a properly functioning aortic prosthesis. There was no significant systolic gradient across the pulmonary valve (PPG = 15 mm Hg). No shunt across the interventricular septum was detected.

A modification of the Le- compte operation is reported in which the pulmonary valve is pre- served. This technique is applicable in certain anomalies of ventriculoar- terial connection with ventricular septal defect and subpulmonary sten- osis. The main advantage of this modification, besides preservation of growth potential of the translocated pulmonary artery, consists of preser- vation of pulmonary valve function, which may have a beneficial effect on the long-term preservation of right ventricular compliance. (Eur J Cardio-thorac Surg (1996) 10: 585-589)

Congenital pulmonary valve disease is often not discovered until adolescence or adulthood. Transthoracic two-dimensional echocardiography can provide detailed information regarding right ventricular outflow anatomy, although images are often less satisfactory than those obtained in infants and children. The more recent addition of biplanar transesophageal echocardiography has enhanced our ability to image the right ventricular outflow tract, pulmonary valve, and pulmonary artery noninvasively. Pulsed and continuous-wave Doppler estimates of subvalvular and transvalvular gradients have proved to be accurate. Doppler color flow mapping has proved useful in determining the location and direction of stenotic and regurgitant flow. With no accepted standard for comparison, quantification of regurgitation remains problematic. In many cases, echocardiography has replaced catheterization and angiography in the evaluation and long-term follow-up of congenital pulmonary valve disease before and after intervention.

A male infant with tetralogy of Fallot was given a modified left Blalock-Taussig shunt at 11 days of age because of duct dependence. Twenty months later, because of increasing cyanosis and polycythaemia, he was given palliative treatment with balloon dilatation of the right ventricular outflow tract. Seven months later a right ventricular angiogram showed further progression of the infundibular stenosis with poor anterograde opacification of the pulmonary arteries. He underwent percutaneous transcatheter myectomy of the infundibulum with an atherectomy catheter. One month later he was well and less cyanosed.

A case of severe pulmonary valvar stenosis and infundibular obstruction has been reported. Infundibular obstruction was so severe that no catheter could be advanced into the pulmonary artery. Propranolol, 0.5 mg given intravenously, reduced the obstruction and allowed the balloon dilatation of the pulmonary valve to be carried out without complication. Subsequently oral propranolol helped to remove the infundibular obstruction. We strongly recommend the use of propranolol when infundibular obstruction is present prior or after the balloon pulmonary valvuloplasty.