[Mitochondrial disease and complete heart block. Kearns-Sayre syndrome. Description of a case]. / Malattie mitocondriali e blocco cardiaco completo. Sindrome di Kearns-Sayre. Descrizione di un caso.

Defects of the mitochondrial respiratory chain form a clinically and biochemically heterogeneous group of diseases. Mitochondrial diseases include myopathies and multisystem disorders that are defined either by biochemical abnormalities of the mitochondria or by the presence of "ragged red fibers" in muscle-biopsy specimens stained with modified Gomori's trichrome stain. Several syndromes have been identified. Typical Kearns-Sayre syndrome is a sporadic condition that is characterized by an onset before the age of 20, progressive external ophthalmoplegia, pigmentary retinopathy and cardiac disorders. Mitochondrial DNA deletions were found in patient with Kearns-Sayre syndrome. We report the case of a 33 year-old woman, with neuromuscular syndrome of the Kearns-Sayre type, insulin-sensitive diabetes and complete heart block, who was implanted a pacemaker.

[Primary malignant lymphoma of the heart. Description of a case and review of the literature]. / Linfoma maligno primitivo del cuore. Descrizione di un caso e revisione della letteratura.

Primary cardiac tumors are rare. Approximately 25% of primary cardiac tumors are malignant, with the majority of these being sarcomas. Primary lymphoma of the heart is a very rare malignancy, usually recognized at autopsy or fatal within a few weeks of diagnosis. we report the case of a patient with diffuse large uncleaved cell lymphoma of the heart who had dyspnea, distention of the neck veins, edema of the face and arms. The diagnosis in this patient was aided by 2D-echocardiography, CT scan of the chest and superior vena caval angiography. The diagnosis was confirmed at operation and by histological examination. Surgical procedures were only palliative and aimed at prolonging life. However, prognosis remained severe and unchanged.

[Considerations on myocardial scintigraphy with 201thallium after stress]. / Considerazioni sulla scintigrafia miocardica con 201tallio dopo sforzo.

An heterogeneous group of 12 patients has been studied with 201thallium myocardial scintigraphy after stress (MSS); has been used an electronic calculator which made it possible to increase the reliability of the method. Eight patients were males, 4 females; the average age were 52.6 years. The 201thallium (1.7-2 mCi) is injected (through a Teflon cannula inserted in the forearm) 1' before the interruption of the stress test on the cycloergometer. After 10' tests are made in AP-LAO and LL view; a second test is carried out after an hour, and, if necessary, after 4-6 hours time. The results are interpreted as: a) positive: absence (++) or obvious defect in perfusion (+); b) negative: normal perfusion (-); c) questionable: activity not significantly reduced (+/-). In some cases, the authors studied correlations and discordances between MSS and clinical, coronarographic and echocardiographic data. It has been considered moreover, the uptake's and release's variations of the radio-isotope in relation with experimental knowledge of myocardial extraction and clearance. We conclude by emphasizing the interest of this method and suggesting some practical considerations: 1) Patients with negative MSS don't need coronarography. 2) Positive MSS, but restricted deficit, good delayed reperfusion, good clinical pharmacological control would permit a delay of coronarography. 3) Clear multiple deficit need coronarography. 4) Usefulness of the MSS for long-term control of the evolution of coronary disease and of patients operated of aortocoronary bypass.

[Considerations on myocardial scintigraphy with 201thallium after stress (author's transl)]. / Considerazioni sulla scintigrafia miocardica con 201tallio dopo sforzo.

An heterogeneous group of 24 patients has been studied with 201Thallium myocardial scintigraphy after stress (MSS); has been used an electronic calculator which made it possible to increase the reliability of the method. Nineteen patients were males, 5 females; the average age were 51.7 years S.D. = +/- 7.9. The 201Thallium (2 mCi) is iniected through a Teflon cannula inserted in the forearm l' before the interruption of the stress test on the cycloergometer. After 10' tests are made in AP-LAO and LL view; a second test is carried out after an hour, and, if necessary, after 4-6 hours time. The results are interpreted as: a) positive: absence (++) or obvious defect in perfusione (+); b) negative: normal perfusion (-); c) questionable: activity not significantly reduced (+/-). In some cases, the Authors studied correlations and discordances between MSS and clinical, coronarographic and echocardiographic data. Has been considered moreover, the uptake's and release's variations of the radio-isotope in relation with experimental knowledge of myocardial extration and clearance. We conclude by emphasising the interest of this method and suggesting some practical consideration: 1) patients with negative MSS don't need coronarography; 2) positive MSS, but restricted deficit, good delayed reperfusion, good clinical pharmacological control would permit a delay of coronarography. 3) clear multiple deficit need coronarography; 4) usefulness of the MSS for long-term control of the evolution of coronary disease and of patients operated of aorto-coronary bypass.