Reversal of Left Ventricular Function by PVC Ablation in Dilated Cardiomyopathy Patient

Premature ventricular complex (PVC) usually follows a benign course and shows good response to medical therapy. However, high burden of PVC deteriorates cardiac function and is often associated with progression into dilated cardiomyopathy (DCMP). We report a case of a young patient who recovered from DCMP after PVC ablation. The patient complained of palpitations and dyspnea on exertion. Holter examination revealed an isolated PVC burden of 29%. Despite intensive medical therapy for more than a year, symptoms aggravated and PVC burden was not diminished on follow-up Holter examination. Furthermore, the echocardiogram revealed deteriorated systolic function as well as left ventricular enlargement, indicating progression into DCMP. Surface electrocardiogram indicated PVC origin in the left ventricular outflow tract. Detailed mapping at the right ventricle and left ventricle outflow tract with the aid of 3-dimensional mapping system, demonstrated PVC origin from the left ventricular outflow tract area, between the right and left coronary cusps. Radiofrequency ablation successfully abolished all ventricular premature beats. Follow-up Holter examination revealed no PVC, and the echocardiogram showed recovery to normal systolic function and chamber size. In conclusion, ablation of PVC should be considered when it does not respond to medical therapy and is associated with deterioration of cardiac function.

2q37 Deletion syndrome confirmed by high-resolution cytogenetic analysis

Chromosome 2q37 deletion syndrome is a rare chromosomal disorder characterized by mild to moderate developmental delay, brachydactyly of the third to fifth digits or toes, short stature, obesity, hypotonia, a characteristic facial appearance, and autism spectrum disorder. Here, we report on a patient with 2q37 deletion presenting with dilated cardiomyopathy (DCMP). Congenital heart malformations have been noted in up to 20% of patients with 2q37 deletions. However, DCMP has not been reported in 2q37 deletion patients previously. The patient exhibited the characteristic facial appearance (a flat nasal bridge, deep-set eyes, arched eyebrows, and a thin upper lip), developmental delay, mild mental retardation, peripheral nerve palsy, and Albright hereditary osteodystrophy (AHO)-like phenotypes (short stature and brachydactyly). Conventional chromosomal analysis results were normal; however, microarray-based comparative genomic hybridization revealed terminal deletion at 2q37.1q37.3. In addition, the patient was confirmed to have partial growth hormone (GH) deficiency and had shown a significant increase in growth rate after substitutive GH therapy. Chromosome 2q37 deletion syndrome should be considered in the differential diagnosis of patients presenting with AHO features, especially in the presence of facial dysmorphism. When patients are suspected of having a 2q37 deletion, high-resolution cytogenetic analysis is recommended.

Dilated cardiomyopathy with Graves disease in a young child

Graves disease (GD) can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP) has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF) of 28%. The thyroid function test (TFT) showed elevated serum T3 and decreased thyroid stimulating hormone (TSH) levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.

Dilated cardiomyopathy with Graves disease in a young child

Graves disease (GD) can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP) has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF) of 28%. The thyroid function test (TFT) showed elevated serum T3 and decreased thyroid stimulating hormone (TSH) levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.

Cardiac Resynchronization Therapy in Infant with Dilated Cardiomyopathy during Extracorporeal Membrane Oxygenator

Although heart transplantation is a final therapeutic option in pediatric patients with dilated cardiomyopathy (DCMP), the shortage of pediatric heart donors is a major obstacle. In adults with DCMP characterized by cardiac dyssynchrony, cardiac resynchronization therapy (CRT) is known to be an effective treatment option. However, there is a lack of evidence on the effectiveness of CRT in infants with DCMP. Several studies have reported improvement in hemodynamics and cardiac performance following CRT in infants with DCMP. Here, we report CRT in an infant with DCMP during extracorporeal membrane oxygenation with 5 months of follow-up.

Massive Cardiomegaly due to Dilated Cardiomyopathy Causing Bronchial Obstruction in an Infant

Dilated cardiomyopathy (DCMP) remains a life threatening disease in young patients and is often difficult to differentiate from myocarditis. Early recognition and treatment of DCMP are crucial for good prognoses in this patient population. The clinical course of patients with DCMP that result in cardiogenic shock varies according to the etiology as well as patient age. The volumetric expansion of the enlarged heart can compress adjacent structures causing a number of related symptoms, especially in infants with soft cartilaginous bronchi. Therapeutic strategies for treating these issues vary according to the type of complication encountered. We report a case of severe DCMP with sudden onset of massive cardiomegaly with heart failure complicated by bronchial obstruction in an infant.

The Evaluation of Cardiac Function in Duchenne Muscular Dystrophy

OBJECTIVE: To evaluate the cardiac function and to explore the importance of the evaluation of cardiac function in patients with Duchenne muscular dystrophy (DMD). METHOD: Thirty-nine patients with DMD without any symptoms of heart problems underwent physical examinations and cardiac monitoring including the arterial carbon dioxide (CO2) screening. Thirty one patients underwent pulmonary function test. RESULTS: Among 39 patients 27 showed abnormal electrocardiographic findings such as ventricular hypertrophy, ischemic change, atrial hypertrophy, T wave inversion, sinus tachycardia and ST elevation. 24 patients showed abnormal echocardiographic findings such as abnormal ejection fraction, dilated cardiomyopathy (DCMP), filling abnormality of left ventricle, global hypokinesia and reduced systolic function. 17 patients showed low ejection fraction (below 59%) and 4 of them were diagnosed as DCMP. There were significant correlations between age and ejection fraction (r= 0.552, p<0.01), between functional level and ejection fraction (r= 0.607, p<0.01) and between vital capacity and ejection fraction (r=0.547, p<0.01). However, ejection fraction showed no significant correlations with arterial CO2. CONCLUSION: Routine evaluation of the cardiac function, at least from 10 years of age, and proper treatment following early diagnosis of heart problems were necessary in patients with DMD, because they possibly have been severely affected by cardiac problems without any clinical symptoms.

The role of electrocardiogram in predicting the prognosis of idiopathic dilated cardiomyopathy / 대한내과학회지

BACKGROUND: Although electrocardiographic manifestations of idiopathic dilated cardiomyopathy (DCMP) are usually nonspecific, several studies have suggested that electrocardiogram (ECG) might be used to predict the prognosis. METHODS: The present study was performed to determine the role of standard 12-lead ECG variables as a prognostic factor of patients with idiopathic DCMP. We retrospectively analyzed the ECG findings at the time of the diagnosis in 89 patients with DCMP during a mean follow-up period of 53.2+/-37.1 months. RESULTS: Twenty-eight (31.5%) of the 89 patients died and the cumulative survival rate was 87% at 2 years and 68% at 5 years. By univariate life table analysis, premature ventricular contraction, left bundle branch block, and age were proved as significant predictors. Multivariate analysis using Cox proportional hazards model identified premature ventricular contraction (p=0.014) and left bundle branch block (p=0.02) as an independent predictor for cardiovascular mortality in DCMP. The presence of a premature ventricular contraction increased the mortality 2.8 times and left bundle branch block 2.6 times. CONCLSUION: The present study demonstrates that independent ECG predictors for prognosis of idiopathic DCMP are premature ventricular contraction and left bundle branch block and ECG may be useful in predicting the prognosis of idiopathic dilated cardiomyopathy.

Partial Left Ventriculectomy as a Bridge to Cardiac Transplantation in a Patient of End-Stage Heart Failure: Case Report / 대한흉부외과학회지

A 40-years-old male with dilated cardiomyopathy(DCMP) and end-stage heart failure had undergone partial left ventriculectomy(PLV) in July 1997 and then underwent cardiac transplantation in January 1999. Three months later he showed increased ejection fraction (EF) from 26% to 42.6%, decreased left ventricular end diastolic diameter(LVEDD) from 71mm to 45mm, cardiac output(CO) 3.95 L/min and cardiac index(CI) 2.28 L/min/m2 echocardiographically. Eight months later, left ventriclular end diastolic and systolic diameters increased to 56 and 51 mm respectively and EF decreased to 17% in echocardiographic follow-up. He had been on maximum medication until he underwent cardiac transplantation 18 months after the PLV. Consecutive myocardial biopsies (1, 3 and 6 month later) showed ISHLT (international society of heart and lung transplantation) class 1a and the treatment for rejection was not needed until now on. We report a partial left ventriculectomy as a successful bridge to cardiac transplantation in a patient with DCMP and end-stage heart failure.

Comparison of Coronary Flow Reserve According to The Degree of Hypokinesia in Dilated Cardiomyopathy With Regional Asynergy

BACKGROUND AND OBJECTIVES: Dilated cardiomyopathy(DCMP) is a primary myocardial disease of unknown cause characterized by left ventricular or biventricular dilatation and impaired myocardial contractility. In 1973, Kreulen et al. classified DCMP into two groups-one with generalized hypokinesia and the other with regional asynergy in addition to generalized hypokinesia. Diminished coronary flow reserve has been reported in DCMP with generalized hypokinesia but its mechanism remains obscure. The aim of this study was to investigate the relationship between the degree of microvascular dysfunction and the difference of regional wall motion abnormality in DCMP with regional asynergy. METHODS: The subjects of this study were 11 patients (M:F=:5, mean age:60 15yrs) a diagnosis of DCMP with regional asynergy, normal sinus rhythm without left bundle branch block and normal coronary angiogram who underwent Doppler wire from September 1997 to December 1999. Left ventricle was divided into three territories according to the coronary arterial distribution by echocardiography(A: coronary artery territory showing regional asynergy, I: coronary artery territory showing intermediate wall motion, P: coronary artery territory showing relatively preserved wall motion). Coronary flow reserve(CFR) was measured at the mid portion of left anterior descending artery(LAD), left circumflex artery(LCX) and right coronary artery(RCA) with 0.014 inch Doppler guide wire before and during intracoronary injection of 12-18 of adenosine. Relative coronary flow reserve(rCFR) was obtained by the ratio of the CFR in coronary artery of the territory showing regional asynergy and relatively preserved wall motion to the CFR in coronary artery of the territory showing intermediate wall motion (CFRA/CFRI, CFRP/CFRI). RESULTS: Regional asynergy was observed in LAD territory in 4(36%) patients, LCX territory in 4(36%) patients, RCA territory in 3(28%) patients (p=s). The mean CFR was 2.5 0.6 in LAD, 2.4 0.5 in LCX, 2.4 0.6 in RCA(p=s). The mean CFR and rCFR in coronary arteries showing regional asynergy were significantly lower than those in coronary arteries showing relatively preserved wall motion(2.1 0.5 vs 2.7 0.6, p<0.05, 0.84 0.12 vs 1.11 0.11, p<0.001). CONCLUSION: Degree of regional hypokinesia in DCMP with regional asynergy seems to be associated with that of microvascular dysfunction.

Left ventricular function after mitral valve operation in congenital mitral regurgitation

BACKGROUND: Severe mitral regurgitation is a common clinical entity that can lead to progressive, irreversible left ventricular dysfunction, and thus should be corrected in proper stage of life. Authors have conducted this investigation to assess left ventricular function after mitral valve operation and to determine the predicting factors. METHODS AND RESULTS: The echocardiographic parameters, specifically left ventricular ejection fraction, shortening fraction, end-systolic dimension and volume, and end-diastolic dimension and volume were measured in preoperative and postoperative period of congenital mitral regurgitation patients (n=60), between March 1992 and March 1998. After correction of severe mitral regurgitaion, left ventricular ejection fraction and shortening fraction decreased significantly (p<0.001 and p<0.05 respectively). Furtheremore, after reoperation of recurred mitral regurgitation, left ventricular ejection fraction and shortening fraction decreased significantly (p<0.05). Left ventricular ejection fraction and shortening fraction in mitral valve reoperation group (n=23) is significantly lower than those in non-reoperation group (n=37) in both preoperative and postoperative period (p<0.05). Left ventricular ejection fraction and shortening fraction is also significantly lower in mitral valve replacement group (n=20) than in mitral valvuloplasty group (n=40)(p<0.05). Severe postoperative left ventricular dysfunction led to dilated cardiomyopathy in 5 patients. Postoperative left ventricular end systolic dimension increased significantly in reoperation group and DCMP group respectively (p<0.05). CONCLUSION: After surgical correction of mitral regurgitation, left ventricular dysfunction is frequent and carries a poor prognosis. Postoperative left ventricular dysfunction can be predicted by preoperative ejection fraction, shortening fraction and systolic diameter. Therefore surgical therapy before the onset of left ventricular dysfunction is recommended.

Changes of Mitral Inflow According to Position in Patients with Dilated Cardiomyopathy

BACKGROUND AND OBJECTIVES: Dilated cardiomyopathy(DCMP) probably is the end result of myocardial damage produced by various causes and shows various clinical manifestations. Some patients with DCMP experience more shortness of breath when change their position especially in left lateral decubitus position. We investigated whether the symptomatic changes according to position in DCMP patients were related to the changes of mitral inflow pattern. MATERIALS AND METHOD: DCMP patients with New York Heart Association functional class III or IV, who felt more shortness of breath when changed their position, were studied. The patients with atrial fibrillation or with moderate and severe valvular heart disease were excluded. Early and late atrial left ventricular filling velocities and time velocity integrals(TVI) and it's ratios of mitral inflow, heart rates, isovolumic relaxation time(IVRT), decelaration time(DT) of early mitral inflow were analysed in each decubitus position. RESULTS: 4 men and 3 women were included and their mean age was 60.3 years. 6 patients had mild mitral regurgitation and 4 patients had mild aortic regurgition. All patients felt more shortness of breath in left lateral decubitus position. Peak velocity and TVI of early mitral inflow were not changed significantly according to position. Peak velocity of late mitral inflow was increased significantly with right lateral decubitus position(Lt; 39.0+/- 14.1cm/sec, Rt; 49. 4+/-23.6cm/sec, p<0.05). TVI of late mitral inflow was increased significantly with right lateral decubitus position(Lt; 2.8+/-1.1cm, Rt; 3.8+/-1.9cm, p<0.05). Ratio of early and late mitral inflow peak velocity was decreased significantly with right lateral decubitus position(Lt; 2.13+ 0.34, Rt: 1.62+/-0.57, p<0.05). Ratio of early and late mitral inflow TVI was decreased significantly with right lateral decubitus position(Lt; 3.13+/-1.49, Rt; 2.13+/-1.32, p<0.01). Heart rate, IVRT, DT were not changed significantly according to position. CONCLUSION: In DCMP patients with symptomatic relief in right lateral position, mitral inflow patterns were changed. And this finding suggests that symptomatic relief may be related to decreased left ventricular filling pressure.

A Case Report of Left Ventricular Remodeling Surgery on End-Stage Dilated Cardiomyopathy / 대한흉부외과학회지

We present a case of 58-year-old female with dilated cardiomyopathy(DCMP) in whom we performed left ventricular(LV) remodeling surgery(Batista operation) to reduce the left ventricle diameter and improve left ventricular function. The patient was admitted September 1996 with heart failure NYHA class IV. There was severe orthopnea and peripheral edema. 2-D echocardiography(Echo) showed DCMP with the ejection fraction(EF) 15%, LV end diastolic dimension(LVEDD) 80mm, mitral regurgitation(MR) grade IV, tricuspid regurgitation (TR) grade II. Preoperative cardiac output(CO) was 1.5L/min and cardiac index(CI) was 1.0 L/min/m2. We proceeded with LV remodeling surgery by resection a part of LV lateral wall between both papillary muscle, from the mitral annulus to the LV apex. Size of resected LV wall was 90 x 100 x 15 mm. At the mean time, mitral valve and tricuspid valve were repaired. Postoperative 2-D Echo showed the EF 37%, LVEDD 50 mm, trivial MR, no TR. CO was 3.5L/min and CI was 2.3 L/min/m2. Her fuctional NYHA class was I.

The Pattern of Pulmonary Venous Flow in Various Heart Disease

To evaluate the influencing factors on pulmonary venous flow(PVF) pattern, we studied the relationship between PVF and left ventricular ejection fracton(EF), mitral annulus motion(MAM) and transmitral flow using pulsed doppler echocardiography in patients with dilated cardiomyopathy(DCMP), acute myocardioal infarction(AMI), left ventricular hypertrophy(LVH) and atrial fibrillation(AE). Ther results were as follows : 1) In the normal controls(13 cases), two forward flow during ventricular systole(VS) and diastole(VD) and one retrograde flow during atrial systole(AS) were observed. The peak velocity of VS, VD and AS flow was 45.9cm/s, 42.8cm/s and -18.3cm/sec, respectively. The peak VS/VD ratio was 1.1. 2) In patients with DCMP(11 cases), (a) compared to the noraml subjects, the peak velocity of VS flow and VS/VD ratio were were significantly reduced(p<0.005 and p<0.001, respectively) and were positively correlated with ejection fraction(r=0.8 and r=0.7, respectively) (b) in 2 DCMP cases with severe mitral regurgitation, systolic retrograde flow was observed in the pulmonary vein instead of forward VS flow. 3) In 12 AMI cases and 7 LVH cases with normal or slightly diminished left ventricular systolic function but with abnormal diastolic function. (a) the peak velocity of VS flow and peak VS/VD ratio were significantly increased(r<0.005 and p<0.01, respectively). (b) the peak velocity of VD flow is positively correlated with transmitral E/A ratio(r=0.8) and the peak VS/VD ratio was positively correlated with transmitral pressure half time(r=0.8). (c) the peak velocity of retrograde AS flow was significantly increased(p<0.001). (d) there was no correlation between doppler parameters of PVF and left ventricular ejection fraction. 4) In patients with atrial fibrillation(10 cases), VS flow was markedly diminished or absent and only VD flow was observed. Also, retrograde AS flow was not observed. These findings suggest that the pattern of PVF is influnced by LVEF, MAM, transmitral inflow and atrial contraction. However, main contributary factors in determining the pattern of PVF in each disease are diverse according to its main pathophysiology.