Clarifying the Anatomy of Tetralogy of Fallot with S-shaped Ascending Aorta.

We recently encountered several cases of tetralogy of Fallot with an abnormally oriented S-shaped ascending aorta. In this retrospective study, we sought to clarify morphology of this unusual under-recognized variant. Databases were reviewed to identify all patients with tetralogy of Fallot having an S-shaped ascending aorta. Computed tomographic angiography was used for the assessment of cardiac morphology. Out of the 21 patients, 18 (86%) had a right aortic arch, 2 (9%) had a left aortic arch, and the remaining patient (5%) had a double aortic arch. Patients with a right aortic arch, compared to age and sex-matched patients with a right aortic arch but normally oriented ascending aorta, had lesser aortic override (29.3 ± 14% vs 54.8 ± 13.2%; p = 0.0001) and a wider ascending aorta (25.2 ± 6.9 vs 18.0 ± 3.2 mm; p = 0.0003). The S-shaped ascending aorta was located posteriorly, with a higher sterno-aortic distance (25.5 ± 7.7 vs 9.9 ± 4.5 mm; p = 0.0001). The ascending aorta among patients with tortuosity was longer (4.12 ± 1.7 vs 3.07 ± 0.82, p = 0.03) but with similar tortuosity index (1.22 ± 0.19 vs 1.15 ± 0.17, p = 0.23). Of the cases with right aortic arch and S-shaped ascending aorta, 16 (89%) had extrinsic compression of the right pulmonary artery (p = 0.0001), while 7 (39%) had crossed pulmonary arteries (p = 0.008), with no such findings among those with normally oriented ascending aorta. Tetralogy of Fallot with an S-shaped ascending aorta is a variant with lesser aortic override and a more posteriorly located ascending aorta. Compression of the right pulmonary artery and crossed pulmonary arteries is frequent in the presence of a right-sided aortic arch. These findings have important implications for optimal diagnosis and surgical repair.

Identifying Challenges and Potential Solutions for Sustainable Kidney Nutrition Care Delivery in Selected Asian Countries.

BACKGROUND: Recent surveys highlight gross workforce shortage of dietitians in global kidney health and significant gaps in renal nutrition care, with disparities greater in low/low-middle income countries. OBJECTIVE: This paper narrates ground experiences gained through the Palm Tocotrienols in Chronic Hemodialysis (PaTCH) project on kidney nutrition care scenarios and some Asian low-to-middle-income countries namely Bangladesh, India, and Malaysia. METHOD: Core PaTCH investigators from 3 universities (USA and Malaysia) were supported by their postgraduate students (n = 17) with capacity skills in kidney nutrition care methodology and processes. This core team, in turn, built capacity for partnering hospitals as countries differed in their ability to deliver dietitian-related activities for dialysis patients. RESULTS: We performed a structural component analyses of PaTCH affiliated and nonaffiliated (Myanmar and Indonesia) countries to identify challenges to kidney nutrition care. Deficits in patient-centered care, empowerment processes and moderating factors to nutrition care optimization characterized country comparisons. Underscoring these factors were some countries lacked trained dietitians whilst for others generalist dietitians or nonclinical nutritionists were providing patient care. Resolution of some challenges in low-to-middle-income countries through coalition networking to facilitate interprofessional collaboration and task sharing is described. CONCLUSIONS: We perceive interprofessional collaboration is the way forward to fill gaps in essential dietitian services and regional-based institutional coalitions will facilitate culture-sensitive capacity in building skills. For the long-term an advanced renal nutrition course such as the Global Renal Internet Course for Dietitians is vital to facilitate sustainable kidney nutrition care.

Pulmonary arterial compliance in patients of CHD with increased pulmonary blood flow.

INTRODUCTION: Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the pulmonary arterial compliance in patients with left to right shunt lesions and its utility in clinical decision-making. MATERIALS AND METHODS: In this single-centre retrospective study, we reviewed cardiac catheterisation data of consecutive patients of left to right shunt lesions catheterised over one year. In addition to the various other parameters, pulmonary arterial compliance was calculated, as indexed pulmonary flow (Qpi) / (Heart rate × pulse pressure in the pulmonary artery). RC time was also calculated, as the product of pulmonary arterial compliance and pulmonary vascular resistance index. Patients were divided into "operable," "borderline," and "inoperable" based on the decision of the treating team, and the pulmonary arterial compliance values were evaluated in these groups to study if it can be utilised to refine the operability decision. RESULTS: 298 patients (Median age 16 years, 56% <18 years) with various acyanotic shunt lesions were included. Overall, the pulmonary arterial compliance varied with Qpi, pulmonary artery mean pressure, and pulmonary vascular resistance index, but did not vary with age, type of lesion, or transpulmonary gradients. The median pulmonary arterial compliance in patients with normal pulmonary artery pressure (Mean pulmonary artery pressure less than 20 mmHg) was 4.1 ml/mmHg/m2 (IQR 3.2). The median pulmonary arterial compliance for operable patients was 2.67 ml/mmHg/m2 (IQR 2.2). Median pulmonary arterial compliance was significantly lower in both inoperable (0.52 ml/mmHg/m2, IQR 0.34) and borderline (0.80 ml/mmHg/m2, IQR 0.36) groups when compared to operable patients (p < 0.001). A pulmonary arterial compliance value lower than 1.18 ml/mmHg/m2 identified inoperable patients with high sensitivity and specificity (95%, AUC 0.99). However, in borderline cases, assessment by this value did not agree with empirical clinical assessment.The median RC time for the entire study population was 0.47 S (IQR 0.30). RC time in operable patients was significantly lower than that in the inoperable patients (Median 0.40 IQR 0.23 in operable, 0.73 0.25 in inoperable patients (p < 0.001). CONCLUSIONS: Addition of pulmonary arterial compliance to the routine haemodynamic assessment of patients with shunt lesions may improve our understanding of the pulmonary circulation and may have clinical utility.

Early repolarization syndrome, epilepsy, and atrial fibrillation in a young girl with novel KCND3 mutation managed with quinidine.

A 6-year-old girl presented with a difficult to control epilepsy syndrome. On evaluation, additional presyncope episodes associated with polymorphic ventricular tachycardia were also noted. A diagnosis of early repolarization syndrome (ERS) was made with an early repolarization pattern on electrocardiogram, documented VT episodes, and clinical presyncope (proposed Shanghai score 7). Paroxysmal atrial fibrillation (AF) was also noted on 24-h Holter recordings. The child was stabilized with isoprenaline infusion and was later discharged with arrhythmia control on quinidine and cilostazol. The genetic evaluation revealed a potassium channel KCND3 gene missense mutation. The case highlights the association of epilepsy syndrome and AF with ERS; the possible association of KCND3 gene mutation with a malignant phenotype; and management issues in a small child.

Statin and aspirin as adjuvant therapy in hospitalised patients with SARS-CoV-2 infection: a randomised clinical trial (RESIST trial).

BACKGROUND: Statins and aspirin have been proposed for treatment of COVID-19 because of their anti-inflammatory and anti-thrombotic properties. Several observational studies have shown favourable results. There is a need for a randomised controlled trial. METHODS: In this single-center, open-label, randomised controlled trial, 900 RT-PCR positive COVID-19 patients requiring hospitalisation, were randomly assigned to receive either atorvastatin 40 mg (Group A, n = 224), aspirin 75 mg (Group B, n = 225), or both (Group C, n = 225) in addition to standard of care for 10 days or until discharge whichever was earlier or only standard of care (Group D, n = 226). The primary outcome variable was clinical deterioration to WHO Ordinal Scale for Clinical Improvement ≥ 6. The secondary outcome was change in serum C-reactive protein, interleukin-6, and troponin I. RESULTS: The primary outcome occurred in 25 (2.8%) patients: 7 (3.2%) in Group A, 3 (1.4%) in Group B, 8 (3.6%) in Group C, and 7 (3.2%) in Group D. There was no difference in primary outcome across the study groups (P = 0.463). Comparison of all patients who received atorvastatin or aspirin with the control group (Group D) also did not show any benefit [Atorvastatin: HR 1.0 (95% CI 0.41-2.46) P = 0.99; Aspirin: HR 0.7 (95% CI 0.27-1.81) P = 0.46]. The secondary outcomes revealed lower serum interleukin-6 levels among patients in Groups B and C. There was no excess of adverse events. CONCLUSIONS: Among patients admitted with mild to moderate COVID-19 infection, additional treatment with aspirin, atorvastatin, or a combination of the two does not prevent clinical deterioration. Trial Registry Number CTRI/2020/07/026791 ( http://ctri.nic.in ; registered on 25/07/2020).

Transcatheter closure of Abernethy malformation associated with interrupted inferior caval vein and other systemic venous anomalies.

Abernethy malformation is a rare entity. We report a 5-year-old boy presenting with severe pulmonary hypertension in whom Abernethy malformation and inferior caval vein interruption were diagnosed by CT angiography. In addition, the iliac veins were thrombosed with multiple venous collateral drainage. This abnormal venous anatomy caused difficulty in device closure of the Abernethy malformation, which was successfully closed using a vascular plug.

The International Society of Renal Nutrition and Metabolism Commentary on the National Kidney Foundation and Academy of Nutrition and Dietetics KDOQI Clinical Practice Guideline for Nutrition in Chronic Kidney Disease.

The Academy of Nutrition and Dietetics and the National Kidney Foundation collaborated to provide an update to the Clinical Practice Guidelines (CPG) for nutrition in chronic kidney disease (CKD). These guidelines provide a valuable update to many aspects of the nutrition care process. They include changes in the recommendations for nutrition screening and assessment, macronutrients, and targets for electrolytes and minerals. The International Society of Renal Nutrition and Metabolism assembled a special review panel of experts and evaluated these recommendations prior to public review. As one of the highlights of the CPG, the recommended dietary protein intake range for patients with diabetic kidney disease is 0.6-0.8 g/kg/day, whereas for CKD patients without diabetes it is 0.55-0.6 g/kg/day. The International Society of Renal Nutrition and Metabolism endorses the CPG with the suggestion that clinicians may consider a more streamlined target of 0.6-0.8 g/kg/day, regardless of CKD etiology, while striving to achieve intakes closer to 0.6 g/kg/day. For implementation of these guidelines, it will be important that all stakeholders work to detect kidney disease early to ensure effective primary and secondary prevention. Once identified, patients should be referred to registered dietitians or the region-specific equivalent, for individualized medical nutrition therapy to slow the progression of CKD. As we turn our attention to the new CPG, we as the renal nutrition community should come together to strengthen the evidence base by standardizing outcomes, increasing collaboration, and funding well-designed observational studies and randomized controlled trials with nutritional and dietary interventions in patients with CKD.

Interventricular membranous septal aneurysm is seen on multidetector computed tomography in postoperative child.

BACKGROUND: Ruptured interventricular septal aneurysms are rare. We present a case of an interventricular membranous septal aneurysm in a 7-year-old boy who presented with features of congestive heart failure, 2 years after undergoing a patch closure of perimembranous ventricular septal defect and tricuspid valve repair. Material and methods and results: Transthoracic echocardiography suggested the presence of an aneurysm that has ruptured into the right ventricle (RV), however, the precise origin of the aneurysm could not be identified on echocardiography. Subsequently, a computed tomography (CT) angiography was done which revealed that the aneurysm was originating from the membranous interventricular septum with evidence of rupture into the RV inflow. DISCUSSION: Due to its close proximity to the aortic annulus, an aneurysm of the membranous interventricular septum may be confused with a sinus of Valsalva aneurysm, especially if the former is associated with aortic regurgitation. CONCLUSION: This case highlights the importance of CT in the precise differentiation of these two abnormalities.

Pulmonary-to-systemic venous collateral in obstructed supracardiac totally anomalous pulmonary venous connection: Blood finds a way!

We describe a rare variant of obstructed supracardiac totally anomalous venous connection in a 7-day-old boy where the vertical vein was occluded and the entire pulmonary venous return was draining through a large tortuous pulmonary-systemic venous collateral. The case highlights the importance of computed tomography angiography in demonstrating such complex anatomies in congenital heart diseases.

An unusual pediatric case of tuberculosis-associated mediastinal fibrosis with concomitant pulmonary arterial and venous occlusion.

We present a case of a 4-year-old boy with tuberculosis-associated mediastinal fibrosis with concomitant pulmonary arterial and venous occlusion, highlighting the role computed tomography angiography in identification of the disease process, coexistent vascular abnormalities and associated complications.

Anomalies of coronary arteries in tetralogy of Fallot: Evaluation on multidetector CT angiography using dual-source scanner.

OBJECTIVES: The present study sought to determine the prevalence and evaluate the patterns of various anomalies of coronary arteries in patients with tetralogy of Fallot (TOF) on multidetector CT angiography using a dual-source CT scanner. METHODS: We retrospectively reviewed CT angiographies of 955 pediatric patients, diagnosed with TOF between 1st January 2015 and 31st December 2019 and having an optimal evaluation of coronary arteries, with respect to the origin, course, and termination of the coronary arteries and associated cardiovascular anomalies. RESULTS: Anomalies of coronary arteries were observed in 8.27% (79/955) patients with TOF. Origin of accessory/proper left anterior descending (LAD) artery from right coronary artery (RCA) or right coronary sinus was the most common anomalous pattern, seen in 3.14% (30/955) patients, followed by the presence of hypertrophied conal artery seen in 2.62% (25/955) patients. The anomalous coronary artery was crossing the right ventricular outflow tract (RVOT) in all cases where the LAD artery was arising from RCA or right coronary sinus or where RCA was arising from the LAD artery. Coronary arterial anomalies were seen in a significantly higher proportion of patients with an atrial septal defect compared to those without (7/25 [28%] vs. 72/930 [7.74%]; p = .0003). CONCLUSION: The prevalence of anomalies of coronary arteries increases significantly in the presence of TOF as compared to the general population. Majority of these anomalous coronary arteries course anterior to the RVOT which can potentially pose technical difficulties with a risk of adverse surgical outcomes and increased morbidity and mortality, rendering their diagnosis crucial before surgery.

Evaluation of cardiovascular morphology and airway-related abnormalities in tetralogy of fallot with absent pulmonary valve syndrome on multidetector computed tomography angiography.

AIM: To evaluate the cardiovascular, tracheobronchial and pulmonary abnormalities associated with tetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) on multidetector computed tomograpgy (MDCT) angiography. MATERIALS AND METHODS: We conducted a retrospective review of our electronic database from January, 2014 to December, 2020 to identify pediatric patients diagnosed with TOF-APVS on MDCT angiography. Pulmonary anatomy, associated tracheobronchial and coronary compression, compression of intrapulmonary bronchi, lung parenchymal changes and other associated cardiovascular abnormalities were evaluated. RESULTS: Forty-seven patients with TOF-APVS were included in the study. Compression of the airways was observed at multiple levels; 4 (8.5%) patients had carinal compression while right main bronchus and left main bronchus compression was seen in 12 (25.5%) and 16 (34.0%) patients, respectively. Forty-three (91.5%) patients showed abnormal pulmonary arterial branching at the segmental level and compression at the level of intrapulmonary bronchi was seen in 24 (51.1%) patients. Some degree of air trapping was seen in 35 (74.5%) patients whereas atelectasis was observed in 15 (31.9%) patients. Mild coronary arterial compression was seen in 3 (6.4%) patients. CONCLUSION: TOF-APVS is characterized by aneurysmal dilatation of pulmonary arteries leading to a varying degree of airway compression. However, even in the absence of compression of the trachea and main bronchi, there can be distal bronchial compression secondary to abnormal pulmonary arterial branching resulting in lung abnormalities in the majority of these patients. This study highlights the importance of dedicated airway assessment in addition to cardiovascular morphological assessment by MDCT angiography, before planning surgery.

Anomalous branch of right pulmonary artery supplying the left lung: A "pseudo-pulmonary sling".

We report a case of a 9-month-old boy with supracardiac total anomalous pulmonary venous connection showing a small aberrant vessel arising from the right pulmonary artery and traversing below the left main bronchus to supply the anteromedial segment of the left lower lobe.

Effect of acute lower respiratory tract infection on pulmonary artery pressure in children with post-tricuspid left-to-right shunt.

We sought to examine the influence of clinically severe lower respiratory tract infection on pulmonary artery pressure in children having CHD with post-tricuspid left-to-right shunt, as it may have physiological and clinical implications. In a prospective single-centre observational study, 45 children with post-tricuspid left-to-right shunt and clinically severe lower respiratory tract infection were evaluated during the illness and 2 weeks after its resolution. Pulmonary artery systolic pressure was estimated non-invasively using shunt gradient by echocardiography and systolic blood pressure measured non-invasively.Median pulmonary artery systolic pressure during lower respiratory tract infection was only mildly (although statistically significantly) elevated during lower respiratory tract infection [60 (42-74) versus 53 (40-73) mmHg, (p < 0.0001)]. However, clinically significant change in pulmonary artery systolic pressure defined as the increase of >10 mmHg was present in only 9 (20%) patients. In the absence of hypoxia or acidosis, only a small minority (9%, n = 4) showed significant pulmonary artery systolic pressure rise >10 mmHg. In the absence of hypoxia or acidosis, severe lower respiratory tract infection in patients with acyanotic CHD results in only mild elevation of pulmonary artery systolic pressure in most of the patients.

Characteristics of Children with Acute Rheumatic Carditis from a High-Incidence Region: Importance of Unexplained Worsening of Functional Class.

BACKGROUND: Acute rheumatic fever (ARF) and acute rheumatic carditis (ARC) continue to be a major public health problem in developing countries. OBJECTIVE: To study the characteristics of children with ARC being treated at a tertiary centre. METHODS AND RESULTS: We studied 126 children (mean age 10.4 ± 2.3 years, range 5-15 years, 60% males) diagnosed with ARC by treating cardiologists. Most had lower socio-economic status. Fifty of 126 (40%) presented with a first episode of ARC. Joint symptoms were present in 29% and fever in 25%. Only 2.4% had subcutaneous nodules and none had erythema marginatum or chorea. Fifty-one percent presented in NYHA class II and 29% in NYHA class III or IV. Tachycardia and heart failure were present in 53% and 21%, respectively. Recent worsening of NYHA class (dyspnoea) was the commonest feature (48%). Laboratory investigations showed raised antistreptolysin O titres (>333 units) in only 36.7% of patients. Raised C-reactive protein (CRP) was present in 70%, while raised erythrocyte sedimentation rate was found in only 37% of patients. On the basis of above findings, the modified Jones criteria (2015) for the diagnosis of ARF were satisfied only in 46% of children. Echocardiography showed mitral valve thickening in 77% and small nodules on the tip of the leaflets in 43% (27 and 8%, respectively for aortic valve). Left ventricular ejection fraction was <50% in only 3 patients. The dominant valve lesion was mitral regurgitation (MR) (present in 95% of patients; severe in 78%, moderate in 15%), while aortic regurgitation was present in 44% (severe in 14%). CONCLUSIONS: The criteria are often not satisfied by patients being treated for ARC. Recent unexplained worsening of dyspnoea, young age, significant MR, echocardiographic nodules, and elevated CRP are important indicators.

Right superior caval vein to the left atrium in a child with vein of Galen malformation.

Vein of Galen malformation (VGM) is a rare intracranial vascular malformation. High output heart failure is common in infancy and is characterized by dilatation of all the cardiac chambers. We report an unusual case of VGM in a 3-month-old infant without dilatation of the right-sided cardiac chambers. We then demonstrate importance of comprehensive evaluation in detecting rare coexistence of anomalous right superior caval vein connection to the left atrium.

Global Prevalence of Protein-Energy Wasting in Kidney Disease: A Meta-analysis of Contemporary Observational Studies From the International Society of Renal Nutrition and Metabolism.

OBJECTIVE: To better define the prevalence of protein-energy wasting (PEW) in kidney disease is poorly defined. METHODS: We performed a meta-analysis of PEW prevalence from contemporary studies including more than 50 subjects with kidney disease, published during 2000-2014 and reporting on PEW prevalence by subjective global assessment or malnutrition-inflammation score. Data were reviewed throughout different strata: (1) acute kidney injury (AKI), (2) pediatric chronic kidney disease (CKD), (3) nondialyzed CKD 3-5, (4) maintenance dialysis, and (5) subjects undergoing kidney transplantation (Tx). Sample size, period of publication, reporting quality, methods, dialysis technique, country, geographical region, and gross national income were a priori considered factors influencing between-study variability. RESULTS: Two studies including 189 AKI patients reported a PEW prevalence of 60% and 82%. Five studies including 1776 patients with CKD stages 3-5 reported PEW prevalence ranging from 11% to 54%. Finally, 90 studies from 34 countries including 16,434 patients on maintenance dialysis were identified. The 25th-75th percentiles range in PEW prevalence among dialysis studies was 28-54%. Large variation in PEW prevalence across studies remained even when accounting for moderators. Mixed-effects meta-regression identified geographical region as the only significant moderator explaining 23% of the observed data heterogeneity. Finally, two studies including 1067 Tx patients reported a PEW prevalence of 28% and 52%, and no studies recruiting pediatric CKD patients were identified. CONCLUSION: By providing evidence-based ranges of PEW prevalence, we conclude that PEW is a common phenomenon across the spectrum of AKI and CKD. This, together with the well-documented impact of PEW on patient outcomes, justifies the need for increased medical attention.

Nutrition for diabetic retinopathy: plummeting the inevitable threat of diabetic vision loss.

Diabetic retinopathy (DR) is among the leading causes of preventable blindness. Hyperglycemia, hypertension, hyperlipidemia and anemia majorly predispose its pathogenesis. The current treatment modalities of DR include laser photocoagulation therapy, intravitreal corticosteroids, intravitreal anti-vascular endothelial growth factor (VEGF) agents and vitreo-retinal surgery which are costly, highly invasive, unproven for prolonged use and opted in advanced stages of DR. By then retina already encounters a vast damage. Nutrients by their natural physiological, biochemical and molecular action can preserve retinal structure and functions by interfering with the various pathological steps prompting DR incidence, thereby altering the risk of developing this ocular morbidity. Nutrients can also play a central role in DR patients resistant towards the conventional medical treatments. However due to the byzantine interplay existing between nutrients and DR, the worth of nutrition in curbing this vision-threatening ocular morbidity remains silent. This review highlights how nutrients can halt DR development. A nutritional therapy, if adopted in the initial stages, can provide superior-efficacy over the current treatment modalities and can be a complementary, inexpensive, readily available, anodyne option to the clinically unmet requirement for preventing DR. Assessment of nutritional status is presently considered relevant in various clinical conditions except DR. Body Mass Index (BMI) conferred inconclusive results in DR subjects. Subjective Global Assessment (SGA) of nutritional status has recently furnished relevant association with DR status. By integrating nutritional strategies, the risk of developing DR can be reduced substantially. This review summarizes the subsisting knowledge on nutrition, potentially beneficial for preventing DR and sustaining good vision among diabetic subjects.

Aortopulmonary window: Morphology, diagnosis, and long-term results.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades. METHODS: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta. RESULTS: Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up. CONCLUSION: There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival.

Resection of subaortic membrane for discrete subaortic stenosis.

BACKGROUND: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute. METHODS: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken. RESULTS: Median age at surgery was 9.0 years (9 months-47 years). There was one early death. Preoperative peak left ventricular outflow tract (LVOT) Doppler gradient was 83.4 ± 26.2 mmHg (range: 34-169 mmHg). On preoperative echocardiography, aortic regurgitation (AR) was absent in 69 (47.3%), mild in 35 (24%), moderate in 30 (20.5%), and severe in 12 (8.2%). After surgery, the LVOT gradient was reduced to 15.1 ± 6.2 mmHg (P < 0.001). Fourteen patients (9.6%) who had residual/recurrent significant gradients are currently being followed-up or awaiting surgery. There was improvement in AR for operated patients with freedom from AR of 92.6 ± 0.03% at 15 years. Kaplan-Meier survival at 25 years was 93.0 ± 3.9% (95% confidence interval: 79.6, 97.7). Freedom from re-operation at 25 years was 96.9 ± 1.8%. CONCLUSIONS: Long-term results of surgery for discrete SubAM are good. Resection of the membrane along with septal myectomy decreases the risk of recurrence.