Cases reported "Cardiomyopathies"

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1/31. Transvenous pacemaker insertion ipsilateral to chronic subclavian vein obstruction: an operative technique for children and adults.

    subclavian vein occlusion limits insertion of pacing electrodes in children and adults. The concern is greatest in children with a long-term need for pacing systems necessitating use of the contralateral vein and potential bilateral loss of access in the future. We describe an operative technique to provide ipsilateral access in chronic subclavian vein occlusion in five consecutive pediatric (n = 4, mean age 6.5 years) and adult (n = 1, age 70 with bilateral subclavian vein occlusion) patients in whom this condition was noted at the time of pacemaker or ICD implant. Occlusion was documented by venography. Pediatric cardiac diagnoses included complete heart block in all patients, tetralogy of fallot in three, and L-transposition of the great vessels in one. Percutaneous brachiocephalic (innominate) or deep subclavian venous access was achieved by a supraclavicular approach using an 18-gauge Deseret angiocath, a Terumo Glidewire, and dilation to permit one or two 9-11 Fr sheaths. Electrode(s) were positioned in the heart and tunneled (pre- or retroclavicularly) to a pre- or retropectoral pocket. Pacemaker and ICD implants were successful in all without any complication of pneumothorax, arterial or nerve injury, or need for transfusion. Inadvertent arterial access did not occur as compared with prior infraclavicular attempts. One preclavicularly tunneled electrode dislodged with extreme exertion and was revised. Ipsilateral transvenous access for pacemaker or ICD is possible via a deep supraclavicular percutaneous approach when the subclavian venous obstruction is discovered at the time of implant. In children, it avoids the use of the contralateral vein that may be needed for future pacing systems in adulthood. This venous approach provides access large enough to allow even dual chamber pacing in children and can be accomplished safely.
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2/31. Intramyocardial hematoma mimicking abnormal left ventricular trabeculation.

    Intramyocardial hematoma may present as a tumor or pseudoaneurysm on echocardiography. A 68-year-old man was admitted with a subacute posterior wall infarction complicated by ventricular fibrillation. echocardiography showed isolated left ventricular abnormal trabeculations, a finding suggesting an associated skeletal muscle disorder, in the lateral wall. At cardiac surgery, performed 6 weeks later because of severe 3-vessel disease, an intramyocardial hematoma of the lateral wall was excised, and myocardial and skeletal muscle biopsies were taken, which showed neither isolated left ventricular abnormal trabeculations nor skeletal muscle disorder. Postoperatively, echocardiography revealed no abnormal trabeculations.
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3/31. Systemic lupus erythematosus presented as non-inflammatory necrotizing vasculopathy-induced ischemic glomerulopathy and small vessels-related ischemic cardiomyopathy.

    The clinical significance of lupus non-inflammatory necrotizing vasculopathy (NINV) is not well established. For example, since lupus renal NINV is usually reported to coexist with proliferative and active glomerulonephritis, it is difficult to demonstrate the role of NINV on renal pathophysiology. Here we report a 16-year-old SLE boy with renal NINV presenting as ischemic glomerulopathy and small vessels-related ischemic heart failure. The renal biopsy demonstrated mild proliferative glomerulonephritis and NINV initially, and one month later repeated renal biopsy showed NINV with ischemic glomerulopathy. These findings established that NINV, but not proliferative glomerulonephritis, was responsive for his acute renal failure (ARF). Another interesting question is about the pathophysiology of his myocardial dysfunction. This patient presented typical angina and congestive heart failure (CHF). Echocardiograms and ventriculography revealed dilatation of four chambers and low ejection fraction. Serial electrocardiograms demonstrated evolutionary ischemic changes. coronary angiography revealed no abnormality of large vessels. These findings suggested small vascular lesions-induced myocardial ischemia was the underlying mechanism of dilated cardiomyopathy. As myocardial biopsy was not done in our case, we could only speculate, but not prove, that the NINV observed in renal biopsy may also involve in cardiac microvascular beds. Nevertheless, this interesting case emphasized the role of obliterative small vascular lesions in the pathophysiology of ARF and myocardial dysfunction. The patient was treated with high-dose corticosteroid, plasma infusion and hemodialysis. His cardiac function improved gradually, however the renal function did not recover.
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4/31. Primary systemic amyloidosis presenting as angina pectoris due to intramyocardial coronary artery involvement: a case report.

    We describe a 76-year-old Japanese woman with primary systemic amyloidosis who presented with angina pectoris associated with ST-segment and T-wave abnormalities resulting from intramyocardial coronary artery amyloidosis. The patient was admitted to our hospital because of dyspnea and pretibial edema 7 years after the diagnosis of variant angina. A diagnosis of primary systemic amyloidosis (AL amyloid protein) was made after examination of gastric and endomyocardial biopsy specimens. The patient died of progressive, uncontrolled heart failure 3 months later. An autopsy study demonstrated only mild-to-moderate atherosclerosis in the epicardial coronary arteries. However, histological examination of the heart revealed diffuse stenoses and obstructions in the intramural coronary arteries by amyloid deposits. This patient had small-vessel coronary disease with ST-segment changes and angina caused by cardiac amyloidosis. A correct diagnosis of ischemic heart disease due to primary amyloidosis is important for estimation of the prognosis and for appropriate management.
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5/31. Atypical MELAS associated with mitochondrial tRNA(Lys) gene A8296G mutation.

    We report on a unique patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) presenting optic atrophy, cardiomyopathy, and bilateral striatal necrosis before stoke-like episodes became apparent. Skeletal muscle total mitochondrial dna analysis identified a heteroplasmic A to G point mutation in the tRNA(Lys) gene at position 8296. Skeletal muscle pathology revealed typical MELAS findings, including ragged-red fibers cytochrome c oxidase positive strongly succinate dehydrogenase-reactive blood vessels. Recent reports describe the 8296 mutation identified in patients with diabetes mellitus or myoclonus epilepsy with ragged-red fibers, not MELAS. We conclude that the 8296 mutation is likely to be pathogenic and that it may be not only a mutation responsible for diabetes mellitus or myoclonus epilepsy with ragged-red fibers but also for MELAS.
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6/31. Amyloid of heart and lungs in a patient with low output syndrome after coronary artery bypass grafting.

    A 70-year-old patient with two-vessel disease and moderately reduced left ventricular function after a recent myocardial infarction was admitted for a routine coronary artery bypass grafting (CABG) procedure. During anesthetic induction, he became unstable and cardiopulmonary bypass had to be instituted urgently. Despite good bypass flows, the patient died in myocardial failure shortly after surgery. At autopsy, besides severe atherosclerotic coronary artery disease, cardiac and pulmonary amyloidosis were found.
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7/31. Isolated noncompaction of myocardium associated with calcification in the interventricular septum.

    We describe a 12-year-old male with isolated noncompaction of the myocardium and associated abnormal calcification in the basal interventricular septum, and we present a review of the literature. The patient has been healthy and free of symptoms. The electrocardiogram showed abnormal Q waves in III, V1, V2, and ST elevation in V1-V3. exercise testing demonstrated ST depression in V4 and V5. Myocardial scintigraphic examination showed a regional reduction in iodine-1,2,3-beta-methyl-iodophenylpentadecanoic acid uptake in the basal interventricular septum. Since coronary angiography demonstrated normal coronary vessels and the trabeculations were not prominent in this region, we hypothesize that coronary microcirculatory dysfunction may cause subendocardial infarction associated with calcification in the same area.
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8/31. Dynamic cardiomyoplasty in a patient with end-stage cardiomyopathy.

    Dynamic cardiomyoplasty is a relatively new surgical procedure by which a transformed fatigue-resistant skeletal muscle wrapped around the heart is stimulated to contract in synchrony with it, thereby augmenting the ventricular functions of a failing heart. We performed a cardiomyoplasty with latissimus dorsii (LD) in a patient who was refused the heart transplant programme because of pulmonary hypertension and psychosocial contraindications. The patient was 34 years old, functional class grade IV of the new york Heart association (NYHA), with a three-month history, due to ischemic cardiomyopathy with multiple vessels affected, 10% ejection fraction, arteriolar pulmonary resistance of 7.5 U wood. cardiomyoplasty was performed after training the LD muscle for four weeks. One week later the pacemaker was programmed in a DDD mode: amplitude 3.75 V, pulse duration 0.50 ms, AV delay 175 ms. The patient reached functional class grade I-II (NYHA). Inotrope support was discontinued and great clinical improvement was noted. The ejection fraction rose from 10% to 30%. Echocardiographic left ventricular outflow tract velocity increased from 0.33 m/s to 0.60 m/s. These values were compared with radionuclide angiocardiography and echocardiography evaluations. The great clinical improvement and positive changes in left ventricular parameters suggest that cardiomyoplasty is useful in the treatment of some cases of dilated or ischemic cardiomyopathy as an alternative to heart transplantation. Long term follow-up is necessary to evaluate this procedure.
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9/31. The clinical problem of occult cardiac amyloidosis. Forensic implications.

    A 68-year-old man with known coronary heart disease experienced rapidly progressive cardiac dysfunction and was found to have occult cardiac amyloidosis at autopsy. The amyloidosis was undiagnosed during life and initially at autopsy. Marked diffuse involvement of the intramural coronary arteries by amyloid deposits resulted in severe luminal compromise of numerous medium and small vessels. The myocardium proper was virtually spared from amyloid deposits. Amyloid-related coronary narrowing contributed to cardiac ischemia and sudden death. The significance of amyloid coronary disease in this patient relates primarily to the difficulty in considering the diagnosis when other reasons for cardiac signs and symptoms preexist. Also, the adverse effects of amyloid coronary disease may be profound without direct myocardial involvement.
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10/31. A man with saphenous vein graft aneurysms after bypass surgery.

    saphenous vein graft (SVG) aneurysms are a rare complication of coronary artery bypass graft surgery. patients in whom these aneurysms form a fistula with either a cardiac chamber or mediastinal vessel are even more uncommon and present a difficult diagnostic and therapeutic challenge. We present a patient with SVG aneurysms and a fistula to the left atrium.
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