RESUMEN
BACKGROUND: Isolated ventricular noncompaction is considered to predominantly affect the left ventricle. It is characterized by increased left ventricular wall thickness and deep intertrabecular recesses with to-and-fro blood flow that remains in continuity with the ventricular flow. Aim of the study was to present a group of patients with isolated noncompaction of both ventricles mimicking arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS: Reported group consisted of 9 pts initially diagnosed with ARVC (mean age 37.9 y, 7 male), who underwent basic clinical evaluation. CMR was performed in 8 pts, cardiac catheterization in 2 pts and endomyocardial biopsy in 2 pts. Mean age at presentation of first symptoms was 23.5 y (5-44 y). Heart failure symptoms were observed in 4 pts, atrial fibrillation in 3 pts, ventricular tachycardia in 2 pts (polymorphic--in 2 pts) and syncope in 3 pts. Final diagnosis of noncompaction was established according to generally accepted criteria. RESULTS: Morphologic and/or functional changes in the right ventricle were seen in 9 pts (100%): enlargement and hypertrabeculation of the right ventricle in all pts, global hypokinesis in 4 pts, focal wall motion abnormalities and/or bulges typical for ARVC in 5 pts. Two pts had significant tricuspid regurgitation. Endomyocardial biopsy (2 pts) showed abnormal thick endocardium, interstitial fibrosis, myocardial damage and lymphocyte infiltration. CONCLUSIONS: 1) Noncompaction of ventricular myocardium should be considered during the evaluation of right ventricular cardiomyopathies with excessive trabeculation. 2) In problematic cases Task Force criteria for ARVC should be used to improve the accuracy of assessment.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica/diagnóstico , Disfunción Ventricular Izquierda/diagnóstico , Adolescente , Adulto , Anciano , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Disfunción Ventricular Izquierda/fisiopatología , Adulto JovenRESUMEN
AIM: To assess the early results of the pulmonary artery valve transcatheter implantation (PAVTI) in pts included into POL-PAVTI registry. Detailed medical and economic analyses were performed. METHODS: Pulmonary artery valve implantation was performed in 14 pts (9 men), aged 16-31 (mean 24.6 +/- 4.8) years, with pulmonary homograft dysfunction after total repair of tetralogy of Fallot (4 pts), pulmonary atresia (2 pts), pulmonary stenosis (1 pt), common arterial trunk type I (1 pt), Ross procedure (3 pts) and TGA - Rastelli operation (3 pts). Eleven pts underwent in the past 2-5 surgical or/and catheter interventions. Indication for PAVTI was based on clinical evaluation and echocardiographic studies. Assessment of morphological and functional features of the right ventricle (RV) and homograft with the use of cardiac magnetic resonance (CMR) was performed in 10 cases. Pulmonary stenosis (max. pulmonary gradient 32-119, mean 72 +/- 28 mmHg) was observed in 13 pts and/or significant pulmonary regurgitation in 10 pts. The procedure was performed in general anesthesia. The deployment of a valved stent in the pulmonary valve position was preceded by a metal stent implantation. Results were evaluated by echocardiography two days after the procedure and one month later. Four patients were evaluated 6 months after procedure. RESULTS: Time of the procedure varied 60-190 (mean 127 +/- 35) min, time of fluoroscopy ranged 12-31 (mean 21 +/- 11) min. PAVTI was successfully performed in all pts without serious complications. Patients were discharged from the hospital 48-293 (mean 120 +/- 71) h after procedure. Significant reduction of pulmonary gradient after the procedure assessed by echocardiography was observed on the second day (20-60, mean 38 +/- 12 mmHg, p < 0.0001) and one month (19-52, mean 34 +/- 9 mmHg, p < 0.0001). Mild pulmonary regurgitation was observed in 2 pts. In 5 pts evaluated 6 months after procedure haemodynamic parameters were unchanged; no late complications were observed. Average cost of the procedure including a price of the valve (82 000 PLN) was 98 000 PLN. CONCLUSIONS: Pulmonary artery valve transvascular implantation is an effective and safe method of non-surgical treatment for patients with homograft dysfunction. Cost-effectiveness is approvable.
Asunto(s)
Cardiopatías Congénitas/cirugía , Implantación de Prótesis de Válvulas Cardíacas/métodos , Prótesis Valvulares Cardíacas , Arteria Pulmonar/cirugía , Válvula Pulmonar/cirugía , Adolescente , Adulto , Implantación de Prótesis Vascular/métodos , Procedimientos Quirúrgicos Cardíacos/métodos , Ecocardiografía Transesofágica , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico por imagen , Humanos , Masculino , Polonia , Arteria Pulmonar/anomalías , Resultado del Tratamiento , Obstrucción del Flujo Ventricular Externo/cirugía , Adulto JovenRESUMEN
Transcatheter valve replacement has recently been introduced into clinical practice. We present our first experience with non-surgical, transcatheter pulmonary valve implantation in four patients (age 27-31 years, three females) with repaired congenital heart disease who required reintervention to the right ventricular outflow tract due to dysfunction of valve homograft. The Medtronic Melody Transcatheter Pulmonary Valve was successfully implanted in all four patients without complications, releasing the outflow obstruction, and normal function of the biological valve.
Asunto(s)
Cardiopatías Congénitas/terapia , Implantación de Prótesis de Válvulas Cardíacas/métodos , Válvula Pulmonar/cirugía , Obstrucción del Flujo Ventricular Externo/terapia , Adulto , Cateterismo Cardíaco/métodos , Femenino , Cardiopatías Congénitas/complicaciones , Defectos del Tabique Interventricular/terapia , Prótesis Valvulares Cardíacas , Ventrículos Cardíacos/cirugía , Humanos , Masculino , Atención Perioperativa/métodos , Polonia , Diseño de Prótesis , Reoperación , Disfunción Ventricular Derecha/etiología , Disfunción Ventricular Derecha/terapiaRESUMEN
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease associated with fibrofatty tissue replacement in heart muscle leading to arrhythmia, heart failure or sudden death (SCD) often being the first manifestation in probands. At least 50% of cases of ARVC are inherited. AIM: To evaluate costs and cost-effectiveness of diagnosis of the disease in asymptomatic relatives in Poland. METHODS: 239 asymptomatic subjects (mean age 35 years, 120 male) belonging to 42 families affected with ARVC were examined between May 2003 and May 2005. The costs of outpatient visits and additional diagnostic tests were included. Payer perspective was used. RESULTS: In all individuals ECG and transthoracic echocardiography were performed. Magnetic resonance imaging and signal-averaged ECG were performed in 35 patients suspected of having ARVC. The diagnostic criteria for ARVC were fulfilled in 29 patients and 57 subjects were recognised borderline. Total costs of screening amounted to 71 090 PLN (approximately 20,000 euro). The average cost per one case of detected ARVC was 2451 PLN (approximately 680 euro). CONCLUSIONS: Costs of early detection of ARVC in individuals with a family history of the disease in Polish settings are low. Due the avilability of primary prevention of SCD the family screening in asymptomatic subjects is a cost-effective procedure.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica/diagnóstico , Tamizaje Masivo/economía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Displasia Ventricular Derecha Arritmogénica/genética , Cardiomiopatías/diagnóstico , Análisis Costo-Beneficio , Ecocardiografía/economía , Electrocardiografía/economía , Salud de la Familia , Femenino , Humanos , Imagen por Resonancia Magnética/economía , Masculino , Persona de Mediana Edad , PoloniaRESUMEN
AIMS: The ultrastructural features of the myocardium in arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been systematically investigated so far. The recent discovery of gene mutations encoding intercalated disc proteins prompted us to perform a transmission electron microscopy study on endomyocardial biopsies. METHODS AND RESULTS: Twenty-one ARVC probands who fulfilled the international Task Force diagnostic criteria underwent right ventricular endomyocardial biopsy and screening of desmosome (D) protein encoding genes. Myocyte intercalated discs were analysed by transmission electron microscope and the data were compared with those of 10 controls and 10 patients with idiopathic dilated cardiomyopathy. Extensive fibro-fatty replacement with a residual myocardium of 59+/-23% was found in ARVC biopsy samples. Pathogenic D gene mutations were identified in 10 (48%): desmoglein-2 in four, desmoplakin in three and plakophilin-2 in three. Mean D length and D percent length of intercalated disc were significantly higher, D number was significantly lower and D gap was widened in ARVC. Moreover, abnormally located D in 75%, abnormal small junctions in 52%, and pale internal plaques in 32% of ARVC patients were found in the presence of a normal intercalated disc convolution index. CONCLUSION: The ultrastructural evidence of intercalated discs remodelling in ARVC, together with the positive screening of D protein encoding genes in half of probands, are in keeping with an intercellular junction cardiomyopathy.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica/patología , Miocardio/ultraestructura , Adulto , Displasia Ventricular Derecha Arritmogénica/genética , Biopsia/métodos , Cardiomiopatía Dilatada/patología , Desmogleína 2/genética , Desmoplaquinas/genética , Femenino , Humanos , Masculino , Microscopía Electrónica de Transmisión , Mutación/genética , Placofilinas/genética , Estudios Retrospectivos , Factor de Crecimiento Transformador beta3/genética , Remodelación VentricularRESUMEN
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is characterised by fatty and fibrous infiltration of myocardial muscle. Clinical symptoms include dangerous cardiac arrhythmias and heart failure in the advanced form of the disease. ARVD is genetically determined in at least 50% of cases and is characterised by a marked variability of clinical presentation within one family. AIM: To assess the prevalence of the familial form of ARVD in Poland, the mode of inheritance and the risk of sudden cardiac death as well as heart failure development in asymptomatic patients, in whom ARVD was detected during family screening. METHODS: 211 relatives of 40 patients with ARVD were examined. Thirty two families were identified in which at least two members had the disease. The analysed parameters included family history, physical examination, ECG, echocardiography and magnetic resonance. RESULTS: Abnormalities of the right ventricle and/or cardiac arrhythmias suggesting ARVD were found in 71 subjects (mean age 32.4 years). In 28 cases ARVD was diagnosed. From this group, one patient had aborted sudden death. In the remaining 43 subjects a borderline form of the disease was detected. Of this group, one patient died suddenly. The degree of morphological changes in cardiac muscle correlated with patients' age. CONCLUSIONS: 1. The familial form of ARVD is frequent in Poland. 2. ARVD is inherited in an autosomal dominant mode. 3. Sudden cardiac death may be the first symptom of the disease, even in subjects with borderline ARVD. 4. ARVD is a progressive disease. Concomitant left ventricular involvement is not rare and probably represents a late stage of the disease.