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.
Cardiac Complexes, Premature ; Cardiomyopathy, Dilated ; Catheter Ablation ; Deoxycytidine Monophosphate ; Dyspnea ; Electrocardiography ; Follow-Up Studies ; Heart Ventricles ; Humans ; Ventricular Function, Left ; Ventricular Premature Complexes

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.
Autism Spectrum Disorder ; Brachydactyly ; Cardiomyopathy, Dilated ; Chromosome Disorders ; Comparative Genomic Hybridization ; Cytogenetic Analysis* ; Cytogenetics* ; Deoxycytidine Monophosphate ; Diagnosis, Differential ; Eyebrows ; Growth Hormone ; Heart ; Humans ; Intellectual Disability ; Muscle Hypotonia ; Obesity ; Paralysis ; Peripheral Nerves ; Phenotype ; Toes

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.
Adult ; Arrhythmias, Cardiac ; Cardiomegaly ; Cardiomyopathy, Dilated* ; Child* ; Child, Preschool ; Compliance ; Deoxycytidine Monophosphate ; Diarrhea ; Diuretics ; Drug Therapy ; Echocardiography ; Fever ; Follow-Up Studies ; Goiter ; Graves Disease* ; Heart Failure ; Heart Ventricles ; Hepatomegaly ; Humans ; Hyperthyroidism ; Male ; Methimazole ; Methylprednisolone ; Physical Examination ; Pulmonary Edema ; Radiography, Thoracic ; Receptors, Thyrotropin ; Recurrence ; Tachycardia ; Thyroid Function Tests ; Thyrotropin ; Vomiting ; Weight Loss

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.
Adult ; Arrhythmias, Cardiac ; Cardiomegaly ; Cardiomyopathy, Dilated* ; Child* ; Child, Preschool ; Compliance ; Deoxycytidine Monophosphate ; Diarrhea ; Diuretics ; Drug Therapy ; Echocardiography ; Fever ; Follow-Up Studies ; Goiter ; Graves Disease* ; Heart Failure ; Heart Ventricles ; Hepatomegaly ; Humans ; Hyperthyroidism ; Male ; Methimazole ; Methylprednisolone ; Physical Examination ; Pulmonary Edema ; Radiography, Thoracic ; Receptors, Thyrotropin ; Recurrence ; Tachycardia ; Thyroid Function Tests ; Thyrotropin ; Vomiting ; Weight Loss

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.
Adult ; Cardiac Resynchronization Therapy* ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Deoxycytidine Monophosphate ; Extracorporeal Membrane Oxygenation ; Follow-Up Studies ; Heart ; Heart Transplantation ; Hemodynamics ; Humans ; Infant* ; Oxygenators, Membrane* ; Tissue Donors

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.
Bronchi ; Bronchoconstriction ; Cardiomegaly* ; Cardiomyopathy, Dilated* ; Deoxycytidine Monophosphate ; Heart Failure ; Humans ; Infant* ; Myocarditis ; Prognosis ; Shock, Cardiogenic

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.
Carbon Dioxide ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Deoxycytidine Monophosphate ; Early Diagnosis ; Echocardiography ; Electrocardiography ; Heart ; Heart Ventricles ; Humans ; Hypertrophy ; Hypokinesia ; Mass Screening ; Muscular Dystrophy, Duchenne* ; Physical Examination ; Respiratory Function Tests ; Tachycardia, Sinus ; Vital Capacity

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.
Bundle-Branch Block ; Cardiomyopathy, Dilated* ; Deoxycytidine Monophosphate ; Diagnosis ; Electrocardiography* ; Follow-Up Studies ; Humans ; Life Tables ; Mortality ; Multivariate Analysis ; Prognosis* ; Proportional Hazards Models ; Retrospective Studies ; Survival Rate ; Ventricular Premature Complexes

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.
Biopsy ; Cardiomyopathy, Dilated ; Deoxycytidine Monophosphate ; Echocardiography ; Follow-Up Studies ; Heart Failure* ; Heart Transplantation* ; Heart* ; Humans ; Lung ; Male

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.
Adenosine ; Bundle-Branch Block ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Coronary Vessels ; Deoxycytidine Monophosphate ; Diagnosis ; Dilatation ; Heart Ventricles ; Humans ; Hypokinesia*