Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study.

OBJECTIVES: The aim of the present investigation was to redefine the clinicopathologic profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC), with special reference to disease progression and left ventricular (LV) involvement. BACKGROUND: Long-term follow-up data from clinical studies indicate that ARVC is a progressive heart muscle disease that with time may lead to more diffuse right ventricular (RV) involvement and LV abnormalities and culminate in heart failure. METHODS: Forty-two patients (27 male, 15 female; 9 to 65 years old, mean [+/-SD] age 29.6 +/- 18) from six collaborative medical centers, with a pathologic diagnosis of ARVC at autopsy or heart transplantation, and with the whole heart available, were studied according to a specific clinicomorphologic protocol. RESULTS: Thirty-four patients died suddenly (16 during effort); 4 underwent heart transplantation; 2 died as a result of advanced heart failure; and 2 died of other causes. Sudden death was the first sign of disease in 12 patients; the other 30 had palpitations, with syncope in 11, heart failure in 8 and stroke in 3. Twenty-seven patients experienced ventricular arrhythmias (ventricular tachycardia in 17), and 5 received a pacemaker. Ten patients had isolated RV involvement (group A); the remaining 32 (76%) also had fibrofatty LV involvement that was observed histologically only in 15 (group B) and histologically and macroscopically in 17 (group C). Patients in group C were significantly older than those in groups A and B (39 +/- 15 years vs. 20 +/- 8.8 and 25 +/- 9.7 years, respectively), had significantly longer clinical follow-up (9.3 +/- 7.3 years vs. 1.2 +/- 2.1 and 3.4 +/- 2.2 years, respectively) and developed heart failure significantly more often (47% vs. 0 and 0, respectively). Patients in groups B and C had warning symptoms (80% and 87%, respectively, vs. 30%) and clinical ventricular arrhythmias (73% and 82%, respectively, vs. 20%) significantly more often than patients in group A. Hearts from patients in group C weighed significantly more than those from patients in groups A and B (500 +/- 150 g vs. 328 +/- 40 and 380 +/- 95 g, respectively), whereas hearts from both group B and C patients had severe RV thinning (87% and 71%, respectively, vs. 20%) and inflammatory infiltrates (73% and 88%, respectively, vs. 30%) significantly more often than those from group A patients. CONCLUSIONS: LV involvement was found in 76% of hearts with ARVC, was age dependent and was associated with clinical arrhythmic events, more severe cardiomegaly, inflammatory infiltrates and heart failure. ARVC can no longer be regarded as an isolated disease of the right ventricle.

[Electrocardiography of high resolution in the diagnosis of arrhythmogenic dysplasia of the right ventricle]. / Elektrokardiogram o wysokiej rozdzielczosci w rozpoznawaniu arytmogennej dysplazji prawej komory.

UNLABELLED: To assess the diagnostic value of high resolution ECG in ARVD which is a rare and diagnostically difficult disease, cardiac micropotentials were evaluated in 32 pts with ventricular arrhythmias with LBBB pattern and right axis deviation. Typical ECG-, ECHO-, angiographic features of ARVD were found in 9 pts (mean age 36 y, VT 8 pts, MAS-5). Normal right ventricle (NRV) (Echo, Tc99) was seen in 23 pts (mean age 36 y, VT-14 pts, MAS-6). 25 healthy man (mean age 28 y) served as control (C). RESULTS: [table: see text] Morphology of total QRS complex in pts with ARVD, comparing to NRV pts and C, was exceptionally characteristic: it was composed of high frequency notches seen not only as prolonged LP but also within fundamental QRS complex. CONCLUSION: In pts with ARVD beside presence of LP, significantly prolonged TVAT and characteristic notches within QRS complex are observed, therefore the high resolution ECG is a promising method in noninvasive diagnosis of ARVD.

[Increased tension on the sympathetic nervous system in arrhythmogenic right ventricular dysplasia. Participation in etiopathogenesis?]. / Wzmozone napiecie ukladu sympatycznego w arytmogennej dysplazji prawej komory. Udzial w etiopatogenezie?

UNLABELLED: To evaluate the suspected role of sympathetic nerve tone in pathogenesis of arrhythmogenic right ventricle dysplasia (ARVD), noradrenaline (NOR) and adrenaline (AD) plasma levels were determined by radioenzymatic method in 10 pts with ARVD (mean age 36 y, VT-8 pts, syncope-5) at rest and during peak exercise. 9 healthy men (mean age 28 y) served as control. RESULTS: [table: see text] CONCLUSION: Significant increase of norepinephrine serum level during peak exercise suggests essential role of sympathetic nerve tone in pathogenesis of ARVD.

The role of echocardiography in diagnosing double chambered right ventricle in adults.

OBJECTIVE: To evaluate the utility of echocardiography in diagnosing double chambered right ventricle (DCRV) in adults. DESIGN: A retrospective study. SETTING: Department of Congenital Heart Diseases and Department of Cardiac Surgery, Institute of Cardiology, Warsaw, Poland. PATIENTS: 32 patients, mean age 32.5 years, 21 female, and 11 male. INTERVENTION: Transthoracic (TTE) and transoesophageal (TOE) echocardiographic examination in patients with suspected DCRV. MAIN OUTCOME MEASURES: Direct inspection during surgical treatment of 28 patients diagnosed as having DCRV as an isolated lesion or associated with other pathologies. RESULTS: Echocardiography allowed the final diagnosis of DCRV in 26 patients (81%) out of 32 studied. TTE was diagnostic in 5 (15.6%) whereas TOE was diagnostic in 21 of 21 studied by this technique. Of 6 patients with negative TTE, DCRV was identified by cardiac catheterisation in 3 and directly during surgery in the remaining 3. Of 26 patients diagnosed by echocardiography, the anomalous muscle bundle was discrete in 20 (77%) and diffuse in 6 (23%). In 23 patients (88%) right ventricular outflow obstruction was localised low in the right ventricle, and in the remaining 3 (11.5%), the obstruction was localised high in the right ventricle. Abnormal bundles localised high were discrete, bundles localised in the lower part of right ventricle were discrete in 17 (74%) and diffuse in the remaining 6 (26%). DCRV was an isolated lesion in only 2 patients (6.2%). In all subjects surgical inspection confirmed echocardiographic data. CONCLUSIONS: Echocardiography was very useful to diagnose DCRV in adults as well as to identify its anatomic type. Comparing two different approaches, TOE better defines the entire scope of pathology, including estimation of the resultant systolic pressure gradient within the right ventricular cavity.