Biventricular Hypertrophic Cardiomyopathy with Severe Right Ventricular Outflow Track Obstruction / 대한내과학회지

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.

Biventricular Hypertrophic Cardiomyopathy with Severe Right Ventricular Outflow Track Obstruction / 대한내과학회지

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.

The Brachial Ankle Pulse Wave Velocity is Associated with the Presence of Significant Coronary Artery Disease but Not the Extent

BACKGROUND AND OBJECTIVES: Arterial stiffness is well known as an important risk factor for cardiovascular disease. At our institution, we assessed the association between arterial stiffness, as determined by brachial ankle pulse wave velocity (baPWV), and the extent of coronary artery disease (CAD), as detected by conventional coronary angiography (CAG) in patients who visited the outpatient clinic for angina without any previous history of heart disease. In addition, we evaluated if the level of baPWV could predict the revascularization as a clinical outcome. SUBJECTS AND METHODS: On a retrospective basis, we analyzed the data of 651 consecutive patients who had undergone baPWV and elective CAG for suspected CAD between June 2010 and July 2011, at a 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 high density lipoprotein-cholesterol, and hemoglobin A1c 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-, 2- and 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 3 significant CAD groups, although there was a trend toward the positive correlation. CONCLUSION: Although baPWV was an independent predictor of significant CAD, it was neither associated significantly with the extent of CAD nor with the risk of revascularization. Therefore, baPWV has a limited value for portending the severity of CAD in patients with chest pain.

Right Coronary Artery to Left Ventricular Fistula Associated with Infective Endocarditis of the Mitral Valve

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.

Device Closure of a Large Atrial Septal Defect in a Patient with Severe Pulmonary Arterial Hypertension after 1 Year Use of an Oral Endothelin Receptor Antagonist

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.

A Case of a Pulmonary Arteriovenous Malformation With Ebstein's Anomaly

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.

A Case of Hepatopulmonary Syndrome in a Patient with Child-Pugh Class A Liver Cirrhosis / 결핵및호흡기질환

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.

Deep Vein Thrombosis and Pulmonary Embolism in the 8th Week of Pregnancy

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).

Acute Myocardial Infarction after Embolic Occlusion of Left Anterior Descending Artery by Prosthetic Valve Thrombosis: Report of 1 case / 대한흉부외과학회지

Acute myocardial infarction due to coronary occlusion by emboli originating from the prosthetic valve thrombosis is very rare but fatal disease which needs immediate diagnosis and urgent treatment. We report a case of acute myocardial infarction after left anterior descending embolic occlusion in whom had previous mitral valve replacement. Redo valve replacement following the interventional catheterization and antiplatelet therapy lead to successful results.

Two cases of isolated pulmonary valve infective endocarditis complicating ventricular septal defect / 대한내과학회지

Isolated infective endocarditis in the native pulmonary valve is an unusual clinical entity in non-intravenous drug users. Intravenous drug abuse, alcoholism, sepsis, catheter related infections and congenital heart diseases account for the majority of predisposing factors. We report two cases of isolated pulmonary valve infective endocarditis complicating ventricular septal defect (VSD). A 43 year-old male was admitted because of mild fever, pansystolic murmur on the left lower sternal border. Transthoracic and transesophageal echocardiography revealed a large perimembranous ventricular septal defect and vegetations at the pulmonary valve. After the intravenous use of penicillin and gentamicin, patch closure of VSD with resection of vegetations, resection of anomalous muscle bundles on the right ventricle outlet tract and pulmonary valvuloplasty was performed. A 43 year-old female was admitted with spiking fever, dyspnea. Transthoracic and transesophageal echocardiography showed a small perimenbranous ventricular septal defect with pulmonary valve vegetations. Intravenous penicillin and gentamicin were continued for 4 weeks and she is doing well.

Familial Sick Sinus Syndrome

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.

A Case of Isolated Left Main Coronary Ostial Stenosis Due to Acute Angle Take-off with Clockwise Rotation of Coronary Sinus confirmed by MRI Image

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.

A Case of Mitral Regurgitation due to Windsock Deformity with Perforations of the Anterior Mitral Leaflet-a Late Complication of Endocarditis

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.

A Case of Mitral Regurgitation due to Isolated Cleft Mitral Valve

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.

A Case Report on the Inflation Failure of Distal Portion of Inoue Balloon during Percutaneous Mitral Valvuloplasty in a Patient with Rheumatic Mitral Stenosis

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.

Clinical Observation of Myocardial Bridge

BACKGROUND AND OBJECTIVE: A myocardial bridge(MB) is an anatomical arrangement in which an epicardial coronary artery becomes engulfed for a limited segment by myocardial fibers. Although it has generally been felt that most instance of bridge are benign. Recent reports have suggested that MB can be associated with evidence of myocardial ischemia, myocardial infarction, arrhythmia and sudden death. This study investigated clinical characteristics of myocardial bridge and significance of treadmill test(TMT). METHOD: Among 4317 consecutive coronary angiograms performed from November 1995 to June 1999, 52 patients had a myocardial bridge. For the patients with MB, the clinical data, coronary angiography and the results of treadmill tests were reviewed. RESULT: The overall prevalence of myocardial bridge was 1.22%. Stable angina, atypical chest pain, variant angina, AMI were 33(63%), 15(29%), 2(4%), 2(%) cases, respectively. Electrocardiographic finding were normal in 31 cases(59%), ST-T change in 20 cases(38%), OMI in 1 case(3%). Mean systolic stenosis of MB was 54%, Mean length of segment of MB was 11.96 4.96mm and all patients had MBs of left anterior descending(LAD) coronary artery. Among 23 cases which had been performed TMT, 17 were positive(77%). There was no significant statistical difference between TMT(+) and TMT(-) in clinical characteristic and coronary angiographic data. We divided the patients with MB into two groups [group I(34 cases): systolic compression < 50%(mean 35.1 10.7%), group II(18 cases): systolic compression 50%(mean 63.6 14.7%)] and there were no statistical difference in clinical characteristics, TMT and angiographic data. CONCLUSION: The patients with MB present variable clinical characteristics of stable angina, atypical chest pain, variant angina, acute myocardial infarction. There is no relationship between the degree of systolic compression and TMT positive in MB. We think that symptoms of MB are not developed only by mechanical compression but concerned with other variable mechanism.

Mitral-aortic Intervalvular Fibrosa Pseudoaneurysm with Dual Fistula that Occurred in Prosthetic Aortic Valve Endocarditis

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.

A Case of Isolated Left Main Coronary Ostial Stenosis due to Acute Angle Takeoff

Isolated and severe left main coronary ostial stenosis is a rare case. In the majority of these patients ostial stenosis was 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 young female patient was presented with exertional and stabbing anterior chest pain. There was no history of syphilis, diabetes mellitus, hypertension, hyperlipidemia and smoking. Coronary angiogram showed isolated left main coronary ostial stenosis. Transesophageal echocardiography(TEE) showed acute angle takeoff of the left main coronary artery. She underwent surgical angioplasty of coronary ostia with a patch of autologous pericardium. After angioplasty, TEE showed dilatation of left main coronary ostium and her clinical symptom improved.

A Case Report of Inoue Balloon Deformity Recognized during Percutaneous Mitral Valvuloplasty

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 cm2 (by pressure half-time method), and an Echo score of 10 points. The PMV with Inoue balloon 28 mm 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 cm2. 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.

Two cases of ruptured congenital sinus of Valsalva aneurysms dissecting into the interventricular septum in patients with cerebral infarction

Sinus of Valsalva aneurysms are rare cardiac anomalies and are usually caused by the separation of the aortic wall media from the valve ring tissue. These aneurysms frequently rupture into the low-pressure areas like the right ventricle and right atrium, rarely do they rupture into the left atrium, left ventricle, pericardial sac, or pulmonary artery. Cerebral infarction has been reported as a rare complication of unruptured sinus of Valsalva aneurysm. We experienced very rare two cases of Valsalva aneurysms of right coronary sinus dissecting into the interventricular septum in patients with cerebral infarction. In two cases these aneurysms ruptured into the left ventricle. These aneurysms were excised and the defect was closed with autopericardium. At the end of the surgical repair, coaptation was found to be insufficient and aortic valve replacement was undertaken.