Precipitating Factors for Hospitalization with Heart Failure: Prevalence and Clinical Impact Observations from the Gulf CARE (Gulf aCute heArt failuRe rEgistry).

OBJECTIVE: Despite the expanding burden of heart failure (HF) worldwide, data on HF precipitating factors (PFs) in developing countries, particularly the Middle East, are very limited. We examined PFs in patients hospitalized with acute HF in a prospective multicenter HF registry from 7 countries in the Middle East. METHOD: Data were derived from the Gulf CARE (Gulf aCute heArt failuRe rEgistry) for a prospective, multinational, multicenter study of consecutive patients hospitalized with HF in 47 hospitals in 7 Middle Eastern countries between February 2012 and November 2012. PFs were determined by the treating physician from a predefined list at the time of hospitalization. RESULTS: The study included 5,005 patients hospitalized with acute HF, 2,276 of whom (45.5%) were hospitalized with acute new-onset HF (NOHF) and 2,729 of whom (54.5%) had acute decompensated chronic HF (DCHF). PFs were identified in 4,319 patients (86.3%). The most common PF in the NOHF group was acute coronary syndromes (ACS) (39.2%). In the DCHF group, it was noncompliance with medications (27.8%). Overall, noncompliance with medications was associated with a lower inhospital mortality (OR 0.47; 95% CI 0.28-0.80; p = 0.005) but a higher 1-year mortality (OR 1.43; 95% CI 1.1-1.85; p = 0.007). ACS was associated with higher inhospital mortality (OR 1.84; 95% CI 1.26-2.68; p = 0.002) and higher 1-year mortality (OR 1.62; 95% CI 1.27-2.06; p = 0.001). CONCLUSION: Preventive and therapeutic interventions specifically directed at noncompliance with medications and ACS are warranted in our region.

Clinical features, socioeconomic status, management, short and long-term outcomes of patients with acute myocardial infarction: Phase I results of PEACE MENA registry.

BACKGROUND: The Program for the Evaluation and Management of Cardiac Events in the Middle East and North Africa (PEACE MENA) is a prospective registry program in Arabian countries that involves in patients with acute myocardial infarction (AMI) or acute heart failure (AHF). METHODS: This prospective, multi-center, multi-country study is the first report of the baseline characteristics and outcomes of inpatients with AMI who were enrolled during the first 14-month recruitment phase. We report the clinical characteristics, socioeconomic, educational levels, and management, in-hospital, one month and one-year outcomes. RESULTS: Between April 2019 and June 2020, 1377 patients with AMI were enrolled (79.1% males) from 16 Arabian countries. The mean age (± SD) was 58 ± 12 years. Almost half of the population had a net income < $500/month, and 40% had limited education. Nearly half of the cohort had a history of diabetes mellitus, hypertension, or hypercholesterolemia; 53% had STEMI, and almost half (49.7%) underwent a primary percutaneous intervention (PCI) (lowest 4.5% and highest 100%). Thrombolytics were used by 36.2%. (Lowest 6.45% and highest (90.9%). No reperfusion occurred in 13.8% of patients (lowest was 0% and highest 72.7%).Primary PCI was performed less frequently in the lower income group vs. high income group (26.3% vs. 54.7%; P<0.001). Recurrent ischemia occurred more frequently in the low-income group (10.9% vs. 7%; P = 0.018). Re-admission occurred in 9% at 1 month and 30% at 1 year, whereas 1-month mortality was 0.7% and 1-year mortality 4.7%. CONCLUSION: In the MENA region, patients with AMI present at a young age and have a high burden of cardiac risk factors. Most of the patients in the registry have a low income and low educational status. There is heterogeneity among key performance indicators of AMI management among various Arabian countries.

Licorice-induced severe hypokalemia with recurrent torsade de pointes.

A 38-year-old obese woman presented with recurrent polymorphic ventricular tachycardia secondary to persistent hypokalemia necessitating more than 40 DC shocks. All endocrine investigations for hypokalemia were negative with impression of "mysterious hypokalemia." On repeated inquiry, a hidden history of licorice use was elicited causing persistent hypokalemia. The case highlights a life threatening complication of licorice use. In addition, it reiterates the importance of repeated history taking in a patient with undiagnosed hypokalemia and torsade de pointes which avoided a device therapy.

Acquired right atrial-to-left ventricular shunt in an adult patient after several years of a senning procedure for D-transposition of great arteries.

This report describes the case of an 18-year-old post-Senning patient noted to have a residual ventricular septal defect and severe tricuspid regurgitation together with a shunt from the right atrium to the left ventricle detected several years after surgery.

Digoxin toxicity with normal digoxin and serum potassium levels: beware of magnesium, the hidden malefactor.

BACKGROUND: In recent years, digoxin use has been on the decline, with decreased incidence of digoxin toxicity. Hence, digoxin toxicity, when it occurs, remains an elusive diagnosis to emergency physicians. OBJECTIVE: To present a case of digoxin toxicity with normal levels of digoxin and serum potassium, but with severe hypomagnesemia. CASE REPORT: A 66-year-old woman presented with junctional tachycardia and ectopic atrial tachycardia. She was known to have congestive cardiac failure on diuretic therapy. Her serum digoxin level was within the normal range (2.4 nmol/L [normal = 1.9-2.6]) along with a normal serum potassium level (3.9 mmol/L [normal = 3.5-5]). However, there was severe hypomagnesemia (0.39 mmol/L [normal = 0.65-1.25]) precipitating digoxin-induced dysrhythmia, which responded well to intravenous magnesium therapy. CONCLUSION: This case reiterates that digoxin toxicity can occur in patients with normal digoxin and potassium levels, and in such patients, magnesium needs to be checked and treated to prevent potentially life-threatening dysrhythmias.

Isolated non-obstructive accessory mitral valve tissue in an adult mimicking ruptured chordae.

Accessory mitral valve tissue is commonly associated with other congenital heart diseases and is usually detected in children causing left ventricular outflow tract obstruction. We present an adult patient with isolated non-obstructive accessory mitral valve tissue that was mimicking ruptured chordae of the mitral valve. Accessory mitral valve tissue in adults is very rare and can mimick various causes of left ventricular outflow tract obstruction. This patient represents the first case in literature wherein an unobstructive accessory mitral valve tissue simulated a ruptured chordae. This case illustrates that in patients with suspected mitral valve chordae rupture without any mitral regurgitation, this diagnosis should be considered, which can have therapeutic implications.

The Outcome of Ultrathin-Strut Biodegradable Polymer-Coated Sirolimus-Eluting Stents in Coronary Artery Disease Patients - A Feasibility Study.

Background: Drug-eluting coronary stents with ultrathin struts and biodegradable polymers have been shown to reduce inflammation, neointimal proliferation, and thrombus formation, leading to less early and late complications in patients with coronary artery disease as compared to thinner strut and durable polymer second-generation stents. In Oman, currently, second-generation stents are used for all patients. Objective: The purpose of this feasibility study was to evaluate the clinical safety and performance of ultrathin-strut (60 µm) biodegradable polymer-coated sirolimus-eluting stents in an all-comers patient population. Methods: This was a prospective, observational, single-center, and single-arm investigator-initiated study from August 2018 to August 2019. Inclusion criteria: 18 years of age, patients with symptomatic coronary artery disease indicated for percutaneous coronary intervention, and stenting of at least one coronary lesion. All patients were followed clinically or telephonically at 12 months after the index procedure. Results: A total of 88 patients were recruited in the study, but 10 patients were lost to follow-up and hence excluded from the analysis. The overall mean age was 63 ± 13 years and 78% were males. The main comorbid conditions were hypertension (58%), diabetes mellitus (49%), and hyperlipidemia (26%). Fifty-three percent presented with unstable angina or non-ST elevation myocardial infarction (MI), 10% with ST elevation MI, recent MI 16%, 18% with stable angina, and 1.3% in cardiogenic shock. The mean left ventricular ejection fraction of the cohort was 46 ± 14%. Angiographically, Type A lesions were seen in 25%, Type B in 32%, and Type C in 42%. Left anterior descending stenting was done in 44%, right coronary artery in 32%, left circumflex artery in 14%, left main in 5%, and graft stenting in 4%. Device success was 96%. Procedural success was seen in 97% of patients. At 1-year follow-up, 93% were asymptomatic; overall device-oriented clinical events were 6.8% including cardiac death in 2.7%, target-vessel MI in 2.7%, and target-lesion revascularization in 1.3% which all occurred in uncontrolled diabetic patients. Conclusions: At index admission and 1 year, ultrathin-strut biodegradable polymer-coated sirolimus-eluting stent study showed low device-related adverse clinical events which are comparable to published data for the second-generation stents. This feasibility study shows that these stents can be used in all types of stent-indicated patients with added advantages of biodegradable polymer and ultrathin struts. In addition, measures to prevent, diagnose, and control diabetes need to be taken in Oman as this cohort of patients develop ST after stenting.

Development and Validation of R-hf Risk Score in Acute Heart Failure Patients in the Middle East.

Objectives: The Rajan's heart failure (R-hf) score was proposed to aid risk stratification in heart failure patients. The aim of this study was to validate R-hf risk score in patients with acute decompensated heart failure. Methods: R-hf risk score is derived from the product estimated glomerular filtration rate (mL/min), left ventricular ejection fraction (%), and hemoglobin levels (g/dL) divided by N-terminal pro-brain natriuretic peptide (pg/mL). This was a multinational, multicenter, prospective registry of heart failure from seven countries in the Middle East. Univariable and multivariable logistic regression was applied. Results: A total of 776 patients (mean age = 62.0±14.0 years, 62.4% males; mean left ventricular ejection fraction = 33.0±14.0%) were included. Of these, 459 (59.1%) presented with acute decompensated chronic heart failure. The R-hf risk score group (≤ 5) was marginally associated with a higher risk of all-cause cumulative mortality at three months (adjusted odds ratio (aOR) = 4.28; 95% CI: 0.90-20.30; p =0.067) and significantly at 12 months (aOR = 3.84; 95% CI: 1.23-12.00; p =0.021) when compared to those with the highest R score group (≥ 50). Conclusions: Lower R-hf risk scores are associated with increased risk of all-cause cumulative mortality at three and 12 months.

Morbidity and mortality of acute heart failure patients stratified by mitral regurgitation in the Arabian Gulf: Observations from the Gulf acute heart failure registry (Gulf CARE).

This study aimed to evaluate the clinical outcomes of patients with acute heart failure (AHF) stratified by mitral regurgitation (MR) in the Arabian Gulf. Patients from the Gulf CARE registry were identified from 47 hospitals in seven Arabian Gulf countries (Yemen, Oman, Kuwait, Qatar, Bahrain, the United Arab Emirates, and Saudi Arabia) from February to November 2012. The cohort was stratified into two groups based on the presence of MR. Univariable and multivariable statistical analyses were performed. The population cohort included 5005 consecutive patients presenting with AHF, of whom 1491 (29.8 %) had concomitant MR. The mean age of patients with AHF and concomitant MR was 59.2 ± 14.9 years, and 63.1 % (n = 2886) were male. A total of 58.6 % (n = 2683) had heart failure (HF) with reduced ejection fraction (EF) (HFrEF), 21.0 % (n = 961) had HF with mildly reduced EF (HFmrEF), and 20.4 % (n = 932) had HF with preserved EF (HFpEF). Patients with MR had a lower haemoglobin (Hb) level (12.4 vs. 12.7 g/dL; p < 0.001), and a higher prevalence of left atrial enlargement (80.2 % vs. 55.1 %; p < 0.001), cardiogenic shock (9.7 % vs. 7.3 %; p = 0.006) and atrial fibrillation (7.6 % vs. 5.6 %; p = 0.006), and HFrEF (71.0 % vs. 52.6 %; P < 0.001). Multivariable analysis demonstrated that MR was independently associated with increased all-cause mortality at 1-year and 3-month HF rehospitalization [1-year all-cause mortality, adjusted odds ratio (aOR), 1.40; 95 % confidence interval (Cl): 1.13-1.74; p = 0.002; 3-month HF rehospitalization, aOR, 1.26; 95 % Cl: 1.06-1.49; p = 0.009]. In an Arabian Gulf cohort with AHF, concomitant MR was associated with an increased risk of 1-year mortality and 3-months HF rehospitalization.

Clinical Features, Socioeconomic Status, Management, and Outcomes of Acute Heart Failure: PEACE MENA Registry Phase I Results.

INTRODUCTION: PEACE MENA (Program for the Evaluation and Management of Cardiac Events in the Middle East and North Africa) is a prospective registry in Arab countries for in-patients with acute myocardial infarction (AMI) or acute heart failure (AHF). Here, we report the baseline characteristics and outcomes of in-patients with AHF who were enrolled during the first 14 months of the recruitment phase. METHODS: A prospective, multi-centre, multi-country study including patients hospitalized with AHF was conducted. Clinical characteristics, echocardiogram, BNP (B-type natriuretic peptide), socioeconomic status, management, 1-month, and 1-year outcomes are reported. RESULTS: Between April 2019 and June 2020, a total of 1258 adults with AHF from 16 Arab countries were recruited. Their mean age was 63.3 (±15) years, 56.8% were men, 65% had monthly income ≤US$ 500, and 56% had limited education. Furthermore, 55% had diabetes mellitus, 67% had hypertension; 55% had HFrEF (heart failure with reduced ejection fraction), and 19% had HFpEF (heart failure with preserved ejection fraction). At 1 year, 3.6% had a heart failure-related device (0-22%) and 7.3% used an angiotensin receptor neprilysin inhibitor (0-43%). Mortality was 4.4% per 1 month and 11.77% per 1-year post-discharge. Compared with higher-income patients, lower-income patients had a higher 1-year total heart failure hospitalization rate (45.6 vs 29.9%, p=0.001), and the 1-year mortality difference was not statistically significant (13.2 vs 8.8%, p=0.059). CONCLUSION: Most of the patients with AHF in Arab countries had a high burden of cardiac risk factors, low income, and low education status with great heterogeneity in key performance indicators of AHF management among Arab countries.

Impact of type of thrombolytic agent on in-hospital outcomes in ST-segment elevation myocardial infarction patients in the Middle East.

Little is known about the impact of thrombolytic agents on in-hospital outcomes in the Middle East. The objective of this study was to evaluate the impact of thrombolytic agents on in-hospital outcomes in ST-segment elevation myocardial infarction (STEMI) patients in six Middle Eastern countries. Gulf Registry of Acute Coronary Events was a prospective, multinational, multicentre, observational survey of consecutive acute coronary syndrome patients admitted to 65 hospitals in 2006 and 2007. Out of 1,765 STEMI patients admitted to hospitals within 12 h of symptoms onset, 25, 43, and 30% were treated with streptokinase, reteplase, and tenecteplase, respectively. Median age of the study cohort was 50 (45-59) years and majority were males (89%). The overall median symptom onset-to-presentation and median door-to-needle times were 130 min (65-240) and 45 min (30-75), respectively. Streptokinase patients had worse GRACE risk scores compared to patients who received fibrin specific thrombolytics. Academic hospitals and cardiologists as admitting physicians were associated with the use of fibrin specific thrombolytics. After significant covariate adjustment, both reteplase [odds ratio (OR), 0.38; 95% CI: 0.18-0.79; P = 0.009] and tenecteplase (OR, 0.30; 95% CI: 0.12-0.77; P = 0.012) were associated with lower all-cause in-hospital mortality compared with streptokinase. No significant differences in other in-hospital outcomes were noted between the thrombolytic agents. In conclusion, in light of the study's limitations, fibrin specific agents, reteplase and tenecteplase, were associated with lower all-cause in-hospital mortality compared to the non-specific fibrin agent, streptokinase. However, the type of thrombolytic agent used did not influence other in-hospital outcomes.

Impact of evidence-based cardiac medication on short- and long-term mortality in 7,567 acute coronary syndrome patients in the Gulf RACE-II registry.

OBJECTIVE: To evaluate the impact of evidence-based cardiac medications (EBMs) on 1-month and 1-year mortality among discharged acute coronary syndrome (ACS) patients in the Middle East. METHODS: Data were analyzed from 7,567 consecutive ACS patients admitted to 66 hospitals in 6 Middle Eastern countries enrolled in the Gulf RACE II in October 2008 to June 2009. Individual EBMs or concurrent use of the EBM combination consists of an anti-platelet therapy, angiotensin-converting enzyme inhibitor (ACEI) (or angiotensin II receptor blocker (ARB)), ß-blocker, and a statin at discharge, were evaluated. Analyses were performed using univariate and multivariate statistical techniques. RESULTS: The mean age of the cohort was 56 +/- 12 years with 79% being males. 65% of the patients received the concurrent EBM combination at discharge. Aspirin, clopidogrel, statins, b-blockers and ACEIs/ARBs use was 96%, 71%, 95%, 82% and 81%, respectively. 70% of the patients were prescribed both aspirin and clopidogrel concurrently at discharge. Adjusting for demographic, clinical, revascularization, and country characteristics, the multivariable logistic regression models demonstrated no differences in mortality at both 1-month (3.0 vs. 3.6%; p = 0.828) and 1-year (3.5 vs. 3.5%; p = 0.976) between the concurrent EBM combination users and non-users. CONCLUSION: The majority of the ACS patients in the Middle East were prescribed the guideline recommended EBM combination at discharge. However, potential still remains for further optimization of management. Further studies are required to examine the long term effect of concurrent use of the EBM combination on mortality in the region.

Ebstein anomaly in an adult presenting with wide QRS tachycardia: diagnostic and therapeutic dilemmas.

A 51-year-old man presented to the emergency department with sustained hemodynamically unstable wide QRS tachycardia and was revived successfully by immediate direct current (DC) cardioversion. There was evidence of previous open heart surgery, possibly atrial septal defect closure. Transthoracic echocardiography showed severe Ebstein anomaly with severe tricuspid regurgitation, no residual atrial septal defect, but with severe right ventricular dysfunction. Subsequent electrocardiograms showed transient atrial fibrillation with no manifest Wolff-Parkinson-White (WPW) accessory pathway during sinus rhythm. The cause of wide QRS tachycardia in this patient may be WPW related or ventricular tachycardia. This case illustrates the diagnostic and therapeutic dilemmas in patients with wide QRS tachycardia and suspected WPW syndrome. In addition, this case demonstrates that unoperated Ebstein anomaly can present in late adult life with tachyarrhythmias.

Aortic intramural hematoma with rupture and concomitant acute myocardial infarction: diagnostic and therapeutic dilemmas.

Aortic intramural hematoma is a contained aortic wall hematoma without a demonstrable intimal flap. It is similar to aortic dissection, but the pathology and pathophysiology are different. We report a patient with a chronic descending thoracic aortic intramural hematoma presenting with acute rupture and periaortic hematoma along with concomitant acute ST-elevation myocardial infarction that proved to be catastrophic without intervention. We discuss the diagnostic and therapeutic dilemma.

Atrial fibrillation with wide QRS tachycardia and undiagnosed Wolff-Parkinson-White syndrome: diagnostic and therapeutic dilemmas in a pediatric patient.

A 10-year-old girl presented to the emergency department of a regional hospital with 1 episode of generalized tonic-clonic seizures. Postictal monitoring followed by a 12-lead electrocardiogram showed fast atrial fibrillation with intermittent wide QRS regular tachycardia. Immediately following this, her rhythm changed to wide QRS irregular tachycardia without hemodynamic compromise. She was suspected to have ventricular tachycardia and was treated with intravenous amiodarone with cardioversion to sinus rhythm. Subsequent electrocardiogram in sinus rhythm showed typical features of manifest Wolff-Parkinson-White (WPW) accessory pathway. This case illustrates the diagnostic and therapeutic dilemmas in patients with atrial fibrillation, wide QRS tachycardia, and undiagnosed WPW syndrome with antidromic conduction of atrial arrhythmias through the accessory pathway. Furthermore, this case demonstrates that undiagnosed wide QRS tachycardias need to be treated with drugs acting on the accessory pathway, thus keeping in mind underlying WPW syndrome as a possibility to avoid potentially catastrophic events.

Absent left main with anomalous origin of all three coronary arteries from left aortic sinus: a previously undescribed combination of coronary anomalies.

Coronary artery anomalies resulting in all three coronary arteries arising separately from single sinus of Valsalva is very rare. We report a patient with two coincidental coronary anomalies: absent left main with left anterior descending, and left circumflex arteries arising separately from left sinus of Valsalva along with anomalous origin of right coronary artery from left sinus of Valsalva. Computed tomography angiogram and conventional coronary angiogram showed anomalous right coronary artery from left sinus with an interarterial course, but with no significant lesions. However, there was significant stenosis of left anterior descending and circumflex arteries that was treated with angioplasty and stenting. To the best of our knowledge, absent left main with all three major coronary arteries arising separately from left aortic sinus has never been reported previously.

Left-ventricular non-compaction with congenital left ventricular diverticulum.

Left-ventricular non-compaction may be isolated or associated with other cardiac or noncardiac anomalies. Left-ventricular non-compaction associated with left ventricular diverticulum is very rare. We describe a 30 year-old pregnant woman with a long standing diagnosis of biventricular non-compaction in whom a hidden left ventricular apical diverticulum was detected on transthoracic echocardiography. Both these conditions increase the risk of thromboembolism. Additionally, she was also diagnosed to have endocervical adenocarcinoma. This case suggests that a comprehensive echocardiographic examination is mandatory in cases of suspected isolated left-ventricular non-compaction to detect any other associated cardiac or noncardiac anomalies.

Is there an association between Fahr's disease and cardiac conduction system disease?: A case report.

BACKGROUND: Fahr's disease is a rare neurodegenerative disorder of unknown cause characterized by idiopathic basal ganglia calcification that is associated with neuropsychiatric and cognitive impairment. No case of Fahr's disease with associated cardiac conduction disease has been described in the literature to date. The objective of this case report was to describe a young female with various cardiac conduction system abnormalities and bilateral basal ganglia calcification suggestive of Fahr's disease. CASE REPORT: A 19-year-old female was transferred to our hospital for a pacemaker insertion. Her past medical history included cognitive impairment and asymptomatic congenital complete heart block since birth. Her manifestations included cognitive impairment, tremors, rigidity, ataxia, bilateral basal ganglia calcification without clinical manifestations of mitochondrial cytopathy. She also had right bundle branch block, left anterior fascicular block, intermittent complete heart block, atrial arrhythmias with advanced atrioventricular blocks and ventricular asystole manifested by Stokes-Adams seizures, which was diagnosed as epilepsy. CONCLUSIONS: According to our knowledge, this was the first case report of a su spected association between Fahr's disease and isolated cardiac conduction system disease. In addition, this case illustrated that in patients with heart blocks and seizures, a diagnosis of epilepsy needs to be made with caution and such patients need further evaluations by a cardiologist or electrophysiologist to consider pacing and prevent future catastrophic events.

Outcomes of guideline-based medical therapy in patients with acute heart failure and reduced left ventricular ejection fraction: Observations from the Gulf acute heart failure registry (Gulf CARE).

ABSTRACT: This study aimed to report on the use, predictors and outcomes of guideline-based medical therapy (GBMT) in patients with acute heart failure (HF) with reduced ejection fraction of <40% (HFrEF), from seven countries in the Arabian Gulf.Patients with acute HFrEF (N = 2680), aged 18 years or older, and hospitalized February-November 2012 were recruited and data were collected post discharge at 3 months (n = 2477) and 1 year (n = 2418). The use and doses of GBMT were evaluated as per European, American and Canadian HF guidelines. Analyses were performed using multivariate logistic regression. This study was registered at clinicaltrials.gov (NCT01467973).The majority of patients were on dual (39%) and triple (39%) GBMT modalities, 14% received one GBMT medication, while 7.2% were not on any GBMT medications. On admission, 80% of patients were on renin-angiotensin system (RAS) blockers, 75% on b-blockers and 56% on mineralocorticoid receptor antagonists (MRAs), with a small proportion of these patients were taking target doses (RAS blockers 13%, b-blockers 7.3%, MRAs 14%). Patients taking triple GBMT were younger (P < .001), less likely to have comorbidities such as diabetes mellitus (P < .001) and CKD/dialysis (P < .001), less likely to receive in-hospital invasive treatments (P < .001), and more likely to be treated by a cardiologist (P < .001), than patients on a single medication. Patients taking triple GBMT showed significantly reduced all-cause mortality both at 3-months (P = .048), and at 12-months (P = .003), compared to patients taking no GBMT.Triple GBMT prescribing and dosing in patients with HFrEF were suboptimal in the Arabian Gulf. Further studies are required to investigate GBMT utilization and dosing in the outpatient setting.

A rare type of Gerbode defect.

A Gerbode defect is a left ventricle to right atrial communication. The type I defect (direct, acquired) results in a direct shunt through the atrioventricular part of membranous septum, while a type II (indirect, congenital) defect results in an indirect shunt through a perimembranous ventricular septal defect (VSD) and a defect in the septal tricuspid valve leaflet. We report a rare type of Gerbode defect wherein a small perimembranous VSD is completely covered by an elongated sail-like anterior tricuspid leaflet forming an aneurysm and directing the shunt into right atrium.