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Hypertrophic cardiomyopathy (HCM) has diverse pathophysiological and clinical features, according to the extent and severity of the hypertrophy development. Hypertrophy mostly involves the left ventricle and sometimes causes a left ventricular outflow tract obstruction. Right ventricular involvement is less frequent, and even the severe form of a right ventricular outflow tract (RVOT) obstruction by concurrent right ventricular hypertrophy in a patient with HCM is rare. We report a case of biventricular HCM with a clinically, morphologically, and hemodynamically significant RVOT obstruction, which had been treated successfully with surgical myectomy. (Korean J Med 2019;94:119-123)

Background. The use of anticoagulant therapy (ACT) in patients with acute infective endocarditis (IE) remains a controversial issue. Our study attempts to estimate the impact of ACT on the occurrence of embolic complications and the usefulness of ACT in the prevention of embolism in IE patients.Methods. The present authors analyzed 150 patients with left-sided IE. Embolisms including cerebrovascular events (CVE) and the use of ACT were checked at the time of admission and during hospitalization.Results. 57 patients (38.0%) experienced an embolic event. There was no significant difference in the incidence of CVE and in-hospital mortality between patients with and without warfarin use at admission, although warfarin-naïve patients were significantly more likely to have large (>1 cm) and mobile vegetation. In addition, there was no significant difference in the incidence of postadmission embolism and in-hospital death between patients with and without in-hospital ACT. On multivariate logistic regression analysis, ACT at admission was not significantly associated with a lower risk of embolism in patients with IE.Conclusions. The role of ACT in the prevention of embolism was limited in IE patients undergoing antibiotic therapy, although it seems to reduce the embolic potential of septic vegetation before treatment.

The presence of severe pulmonary arterial hypertension (PAH) in patients with atrial septal defect (ASD) is still thought to preclude shunt closure, although there are several reports of good clinical outcomes after vasodilator therapy. We report the case of a young woman with ASD and severe PAH who was able to successfully undergo percutaneous shunt closure following 1 year use of the oral endothelin receptor antagonist, bosentan.

Metabolic syndrome (MS) is associated with faster progression of degenerative aortic stenosis (AS). However, the influence of MS on AS progression and clinical outcomes in patients with bicuspid aortic valve (BAV) has not been studied. A total of 1,640 patients with BAVs were identified at two

Background and Aim. Because B-type natriuretic peptide is a powerful predictor of heart failure, its capability to predict a fatal outcome in stroke might be limited to the cardioembolic stroke subtype. In this study, we attempt to ascertain the difference in the prognostic value of N-terminal pro-B-type natriuretic peptide (NT-proBNP) between cardioembolic and noncardioembolic stroke subgroups.Methods. 410 acute stroke patients were included. According to the presence of a cardioembolic source (CES), there were 221 patients with CES and 189 patients without CES. Logistic regression analysis was performed to ascertain the association between NT-proBNP and 6-month mortality/functional outcome in each group.Results. The mean age of our patients was 67.2 years (range, 18–97 years). NT-proBNP was a multivariate independent predictor of mortality in the CES group alone, whereas it was only a univariate predictor of 6-month mortality in the total patient and non-CES groups with its association disappearing in the multivariate model. In addition, it was only a univariate predictor of good functional outcome in all of the groups.Conclusions. Our data suggest that NT-proBNP can more reliably predict 6-month mortality in patients with cardioembolic stroke than in patients with other stroke subtypes.

Background and Aim: In recent days, intracranial atherosclerotic stenosis (IAS) has been reportedly related to recurrent stroke and mortality in the total patient population with ischemic stroke. However, its impact on the prognosis of the patients with cardioembolic source is not yet established. This study attempted to investigate whether IAS was associated with poor outcome in stroke patients with cardioembolic source. Methods: Overall, 223 patients with acute ischemic stroke and cardioembolic source were included in this study. IAS was defined as ≥50% stenosis on enhanced MRA, and cardioembolic sources were detected by ECG and echocardiography. Follow-up data were obtained from outpatient medical records and/or telephone interviews. Results: Overall, 60 major clinical events causing poor outcome occurred in 58 patients (26.0%). Patients with IAS had significantly higher rates of death and stroke recurrence than those without IAS (p = 0.015 and 0.046 each by log rank test). The multivariate Cox proportional hazards regression analysis showed that IAS was significantly related to the poor outcome (hazard ratio 1.725, 95% confidence interval 1.020-2.920, p = 0.042). Conclusions: In acute ischemic stroke patients with cardioembolic source, IAS may be considered a marker of a high risk of stroke recurrence or death.

A 27-year-old man with bacterial endocarditis of the mitral valve and embolic episodes was bound to have a large right coronary artery fistula communicating with the left ventricle, immediately inferior to the posterior mitral annulus. The perforation of the posterior leaflet and coronary arteriovenous fistula was identified using two-dimensional Doppler echocardiography. The diagnosis was confirmed by coronary angiography, and the patient underwent a successful operation.

Abciximab has a key role in the treatment of patients with acute coronary syndromes undergoing percutaneous coronary intervention; however, an increased risk of bleeding complications is well recognized. We report a case of serious pulmonary hemorrhage after use of abciximab therapy. A definitive indication and treatment guideline should be available to minimize serious bleeding complications. Additionally, respiratory symptoms should be monitored closely for early detection of serious pulmonary hemorrhage in patients receiving abciximab therapy during percutaneous coronary intervention.

A pulmonary arteriovenous malformation (PAVM) is a rare pulmonary vascular anomaly presenting as dyspnea or recurrent epistaxis. Ebstein's anomaly (EA), a congenital cardiac malformation, is also a rare condition. There have been no reports concerning the co-existence of PAVM with hereditary hemorrhagic telangiectasia (HHT) and EA. A 40-year-old woman was admitted with a 2-month history of increasing dyspnea and several years of recurrent epistaxis. On transthoracic echocardiography, she was diagnosed with EA and agreed to undergo surgical treatment. A chest CT angiography showed a 12-mm serpiginous vascular structure suspicious for a PAVM and a liver CT suggested HTT. Although it is unclear whether or not a concurrent PAVM and EA have an embryologic or genetic relationship, we report a case of a PAVM with EA. Further genetic and embryonic studies are needed to identify a possible relationship of the two medical conditions.

Background This study attempts to explore the clinical features, possible mechanisms and prognosis of the neurologic complications in patients with acute aortic dissection (AD). Methods Medical records of 278 consecutive patients with AD (165 with type A and 113 with type B dissection) over 11.5 years were retrospectively analyzed for clinical history, CT findings, neurologic complications and outcome. Neurologic complications were classified into early-onset or delayed-onset complications. Independent t-test or Chi-square test (or Fisher exact test) was used for comparing the different groups. Multivariable logistic regression analysis was performed to determine the independent association between variables. Results The mean age of the included patients (145 male and 133 female) was 59.4 years (range 19–91 years). 41 patients (14.7%) had a neurologic complication, which included 21 with early-onset complication and 23 with delayed-onset complication, including 3 with both. Advanced age and classic type of dissection were independently associated with the neurologic complication in patients with type A dissection. The most frequent manifestation was ischemic stroke (26 patients, 9.4%), followed by hypoxic encephalopathy (9, 3.2%), ischemic neuropathy (5, 1.8%), spinal cord ischemia (5, 1.8%), seizure (2, 0.7%), hoarseness (1, 0.4%) and septic encephalopathy (1, 0.4%). Overall in-hospital mortality was 10.1%, whereas the complicated group had a mortality rate of 43.9%. Renal impairment, pulse deficit, neurologic complication and nonsurgical treatment were independent variables for determining in-hospital mortality in patients with type A dissection. Conclusions The dominance of neurologic symptom in the early stage of AD may make its early diagnosis difficult. Besides chest pain and widened mediastinum in chest x-ray, variable neurologic symptoms including left hemiparesis with asymmetric pulse and hypotension may suggest underlying AD.

The presence of accessory left atrial chordae tendineae inserting into the mitral valve leaflet is extremely rare. Two long and thin accessory chordae tendineae, one arising from the left atrial dome and the other from the inferior interatrial septum, were incidentally identified during corrective surgery for severe mitral regurgitation from A3 prolapse. Triangular resection of the A3 portion of the anterior mitral valve leaflet including the double accessory chordae tendineae and primary repair followed by posterior ring annuloplasty was successfully performed.

Purposes: We assessed the association between arterial stiffness, as determined by brachial ankle pulse wave velocity (baPWV), and the extent of coronary artery disease (CAD) by conventional coronary angiography (CAG) in patients who visited outpatient clinic for angina without any previous history of heart disease. Method: We retrospectively analyzed the data of 583 consecutive patients who had undergone baPWVand elective CAG for suspected CAD between January 2012 and November 2013 in single cardiovascular center. Results: The baPWV was one of the statistically meaningful predictors of significant CAD (diameter of stenosis >50%) in addition to male gender, age, the level of HDL-cholesterol and HbA1c in multivariate analysis. However, baPWV was not the significant predictor of revascularization. When the extent of CAD was classified into following 4 groups; no significant CAD, 1-, 2and 3-vessel disease, there was significant difference of baPWV between the significant and non significant CAD group, but there was no difference of baPWV among the three significant CAD groups, although there was trend toward the positive correlation. Conclusion: Although baPWV was an independent predictor of significant CAD, it was not significantly associated with the extent of CAD.

Hepatopulmonary syndrome (HPS) is characterized by a defect in arterial oxygenation that's induced by pulmonary vascular dilatation in the setting of liver disease. Some studies have shown the relationship between the presence of the HPS and the severity of liver disease, but there are only rare cases of HPS inpatient with Child-Pugh class A liver cirrhosis. We report here on a case of a 58 years-old male who suffered from progressive dyspnea for the previous few years. He was diagnosed with alcoholic liver cirrhosis 5 years previously. There was no significant abnormality on the chest radiograph and transthoracic echocardiography, but the arterial blood gas analysis revealed severe hypoxemia. Contrast-enhanced transesophageal echocardiograpy with agitated saline demonstrated a delayed appearance of microbubbles in the left cardiac chambers. Thus, he was finally diagnosed with HPS. This case suggests that we should consider HPS when a patient with compensated liver cirrhosis has unexplained dyspnea.

A 29-year-old woman in her 8th week of pregnancy was referred to our hospital for swelling in the lower extremities, rapid onset of dyspnea (1 hr) and pre-syncope. Severe right ventricular dysfunction and moderate pulmonary hypertension were detected using 2-dimentional Doppler echocardiography. In addition, left calf vein and proximal thromboses were detected by venous compression ultrasound imaging. After successful thrombolytic treatment, the patient quickly recovered and was discharged from hospital on subcutaneous low-molecular-weight heparin. She delivered a normal, healthy infant at full-term (40 weeks). (Korean Circulation J 2007;37:130-133)

A valvular perforation is a well-known, and common, complication of infective endocarditis that may adversely affect the clinical outcome. However, a ‘windsock’ deformity of the mitral valve, as a delayed presentation of infective endocarditis, affecting the mitral valve alone, is very rare. A 42-year-old man, who underwent a mitral valvuloplasty and annuloplasty six years previously, suddenly developed pulmonary edema. He had also had a previous history of infective endocarditis, dating back three years. A transthoracic echocardiogram revealed a ‘windsock’ deformity of the anterior mitral leaflet (AML), resulting in an acute severe mitral regurgitation. During the operation, the AML was found to have been damaged by the previous endocarditis, resulting in an aneurysmal change of the central scallop, and a rupture of the roof. A mitral valve replacement was successfully performed, and the patient recovered uneventfully. Here, we report a rare case of a ‘windsock’ deformity of the mitral valve, with two perforations as a delayed complication of a healed infective endocarditis. (Korean Circulation J 2003;33 (4):333-337)

This paper presents a family with sick sinus syndrome, spanning three generations and with an autosomal dominant trait. The proband was affected by atrial fibrillation with a slow ventricular rhythm that required a permanent pacemaker. Her three sons were affected with a sinus node dysfunction and one daughter died suddenly at the age of 32 years. A pacemaker was implanted in the proband and her two sons with symptoms related to bradycardia. One of her sons with the pacemaker died of a cerebrovascular accident several months later. We report a family with sick sinus syndrome requiring the implantation of a pacemaker with a review of the literature. (Korean Circulation J 2003;33 (12):1155-1160)

Isolated left main coronary ostial stenosis is a very rare condition. In the majority of cases there are coexisting diseases in multiple coronary vessels. Here, a case of isolated left main coronary ostial stenosis due to an acute angle take-off, with clockwise rotation of the coronary sinus, confirmed by cardiac MRI is presented. A 44-year old female patient presented with an exertional and stabbing anterior chest pain. The patient had no premedical history. A coronary angiogram showed an isolated left main coronary ostial stenosis due to an acute take-off of the left main coronary artery. A cardiac MRI showed an acute angle take-off of the left main coronary artery, with clockwise rotation of the coronary sinus. The patient underwent surgical angioplasty of the coronary ostia, with a patch of autologous pericardium. This acute angle take-off may be due to rotation of the coronary sinus. (Korean Circulation J 2003;33 (5):435-438)

A cleft in the anterior mitral valve leaflet is commonly associated with an atrioventricular septal defect, but an isolated cleft mitral valve is a rare condition. We report a case of a 27-year-old woman with severe mitral regurgitation due to an isolated cleft mitral valve. The echocardiography showed an anterior cleft on the mitral valve, but a normal sized and positioned left ventricular papillary muscle without septal defect. Under the diagnosis of mitral regurgitation due to the isolated cleft mitral valve the patient underwent mitral cleft repair. After surgery, the further echocardiography showed no mitral regurgitation. (Korean Circulation J 2002; 32(10 :902-905

Since Inoue et al introduced a specially designed balloon catheter for percutaneous mitral valvuloplasty (PMV) in 1984, the Inoue balloon catheter has been a popular device for the management of mitral stenosis. During the procedure several fatal complications, such as cardiac tamponade, perforation of a cardiac chamber, atrial septal defect, thromboembolism, mitral regurgitation and death have all been reported in the literature. There have also been several international reports regarding deformities of the Inoue balloon, but few reports in Korea. We recently experienced a case of an inflation failure of the distal portion of the Inoue balloon during a percutaneous mitral valvuloplasty in a 34 year old female patient with a tight mitral stenosis. To the best of our knowledge, a similar deformity of an Inoue balloon has never been reported in Korea. (Korean Circulation J 2002;32(10):906-910)

Isolated and severe left main coronary ostial stenosis is a rare condition. In the majority of these patients, ostial stenosis has been associated with any of the conditions known to involve the coronary ostia. These conditions include syphilitic aortitis, Takayasu’s aortitis, familial hypercholesterolemia, and aortic valve disease. A 34-year old female patient was presented with exertional and stabbing anterior chest pain. There was no history of syphilis, diabetes mellitus, hypertension, hyperlipidemia or smoking. Coronary angiogram showed isolated left main coronary ostial stenosis. Transesophageal echocardiography (TEE) showed acute angle takeoff of the left main coronary artery. The patient underwent surgical angioplasty of the coronary ostia with a patch of the autologous pericardium. Following angioplasty, TEE showed dilatation of the left main coronary ostium and the clinical symptoms improved. (Korean Circulation J 2001;31(4):442-446)

The Iinvolvement of subaortic structures in the aortic valve endocarditis appears more commonly than previously recognized. These subaortic complications are most commonly located in the mitral-aortic intervalvular fibrosa and may be presented as abscess, or as pseudoaneurysm with or without perforation. Perforated pseudoaneurysm can lead to the development of communication between the left ventricular outflow tract and various cardiac chambers, most commonly the left atrium. These complications are related with poor prognosis. Early and precise recognition of these complications is important for optimal treatment. At present, transesophageal echocardiography (TEE has been validated as the technique of choice. We describe a case of infectious pseudoaneurysm of mitral-aortic intervalvular fibrosa featuring the connection of the fistulous simultaneously to the left atrium and aorta. In our case, accurate interpretation of TEE imaging revealing the subaortic structures was not so easy due to interference of both aortic and mitral prosthetic valves. We expect the further development of (Ed-confirming that here you don’t intend, “We expect to further develop” TEE and other imaging modalities to substantially improve the future diagnosis of these undesirable complications. (Korean Circulation J 2001;31(7 :701-706

Since the Inoue balloon was first introduced for percutaneous mitral valvuloplasty (PMV) in 1984, this procedure has come into widespread use because of its effectiveness, simplicity, and reduced exposure to X-ray radiation. It's the procedure’s complications include cardiac tamponade, atrial septal defect, thromboembolism, ventricular perforation, mitral regurgitation, and rarely balloon rupture. We report a case of Inoue balloon deformity during PMV in 62-year old woman with rheumatic mitral stenosis. Echocardiography revealed severe rheumatic mitral stenosis with a valvular area of 0.95 cm (by pressure half-time method), and an Echo score of 10 points. The PMV with Inoue balloon 28mm was performed. We inflated the balloon to 28 mm in diameter first, and to 29 mm second. A bulging deformity with asymmetrical overinflation of one side of both proximal and distal balloon was recognized. A bulging deformity at the proximal part of Inoue balloon after second inflation. Balloon was not ruptured. Following completion of the procedure, the mitral valve area increased to 1.8 cm. Moderate mitral regurgitation (grade II) was newly developed. This may be the first case of asymmetrical one side inflation and focal bulging deformity reported in Korea.(Korean Circulation J 2001;31(8):830-833)

Background and Objective:Percutaneous transluminal coronary angioplasty of chronic total occlusion has been limited by a relatively low success rate and a high restenosis rate. This study investigated procedural outcome, factors predictive of procedural success and safety of coronary angioplasty for chronic total coronary occlusion. Materials and Methods:The study population was composed of 45 lesions attempting PTCA with or without stent implantation for recanalization of chronic total coronary occlusion between January 1997 and July 1999. The clinical and angiographic data of the 45 lesions were reviewed. The results of successful PTCA in 28 lesions were compared with those in 17 lesions whose PTCA was failed. Results: The overall success of balloon angioplasty and stenting was achieved in 28 lesions (62.2%) and did not differ significantly by clinical variables. The most common cause of failure of balloon angioplasty was inability to pass the guide wire across the occlusion (14 of 23 lesions, 61%). Procedural success was more common in patients with occlusions with a tapered entry configuration (77.2% vs. 47.8%, p=0.042), with lesions without side branches (82.3% vs. 50%, p=0.03). Multiple logistic regression analysis identified the absence of side branch (p 60%) of chronic total coronary occlusions can be successfully dilated by balloon angioplasty with or without stent implantation, with a major complication rate of 2.2%. Therefore, with careful patient selection, we need to try the aggressive recanalization for chronic total coronary occlusion. (Korean Circulation J 2000;30(4):416-423)

Background:Ruptured aneurysms of sinus of Valsalva are rare cardiac anomaly. Here, we analyze retrospectively patients operated on at our hospital during the last 10 years. Methods:Seventeen cases of ruptured congenital aneurysm of sinus of Valsalva (female:male=10:7, mean age 33.2±15.2 year) were operated during the period of January 1989 through August 1998. A ruptured aneurysm of the sinus of Valsalva was diagnosed by transthoracic 2D echocardiography and multiplane esophageal echocardiography. The diagnoses were confirmed at operation. The majority (94.1%) arose from the right coronary sinus. The right ventricle was the most common chamber of rupture (76.5%). Ventricular septal defect was associated in 13 patients (76.5%), of which 8 (61.5%) were subarterial. Ventricular septal defect was more common in aneurysms arising from the right coronary sinus (81.2%). Aortic regurgitation was found in 5 patients (29.4%). One patient underwent aortic valve repair and one an arotic valve replacement. Results:There was no early operative death and no recurrence after the initial repair. Postoperative morbidities were few. There was one late sudden cardiac death 3 months post-surgery. In the majority, the long-term follow-up was uneventful. Conclusion:Surgery for ruptured aneurysm of sinus of Valsalva yields gratifying results, and it should be undertaken as soon as the condition is diagnosed. With recent developments, echocardiography may prove a substitute for cardiac catheterization and angiocardiography in future, and surgery could be undertaken with the help of echocardiography alone. (Korean Circulation J 2000;30(2):183-190)

The scimitar syndrome, a rare complex anomaly, is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. The shape of the Turkish curved sword (scimitar) has provided the name of this syndrome. Additional characteristics of this syndrome such as hypoplasia of the right lung and of the right pulmonary arterial tree, anomalous arterial supply of the right lung from the aorta, dextrocardia and bronchial anomalies are common. Recently we experienced a case of scimitar syndrome (adult form) in a 19-year-old woman patient, so we report the case with a brief review of the literature.

Atrial septal aneurysm (ASA was reported as a cause of unknown origin of central or peripheral thromboembo- lism with patent foramen ovale, aortic debris and spontaneous echo contrast. Especially the paradoxical right to left shunt via micro-fenestration on this sac may be the important cause of this embolism. We report a case of 39 year-old female patient who had atrial septal aneurysm with multiple renal infarction. Symptom was improved with aneurysmectomy and patch closure, then long term anticoagulation has been continued. (Korean Circulation J 1999;29( ( ( (8 :844-847