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Biomedical subjects

V F Huckell

Publications and source records attributed to V F Huckell.

11 recordsLinked to original sources
PubMed1992

Atrial paralysis in a patient with Emery-Dreifuss muscular dystrophy.

Emery-Dreifuss disease is a benign X-linked muscular dystrophy characterized by a distinct pattern of muscle weakness, which is of insidious onset and slow progression. It is associated with atrial paralysis that results in sudden death in early adulthood if left untreated. The authors report the documentation of electrical and mechanical silence confined to the atria in a patient with this disease. Electrocardiography and electrophysiological study document the absence of electrical atrial activity, and inability to pace the atria. Hemodynamic studies demonstrate the absence of A waves, and angiography revealed immobility of the atria. This patient has done well following the institution of permanent ventricular pacing. His brother, who also had muscular dystrophy, died a sudden cardiac death at the age of 29 after refusing medical intervention. Emery-Dreifuss muscular dystrophy is particularly worthy of recognition because of the preventable occurrence of sudden death in young patients with an otherwise excellent prognosis. Permanent ventricular pacing is indicated.

Adult↗
PubMed1992

Anomalous origin of the right pulmonary artery: a 19-year follow-up.

Origin of a pulmonary artery from the aorta is a rare congenital defect which usually is fatal if not surgically corrected in infancy. Medical treatment often is unsatisfactory because of progressive heart failure. Described is a 19-year follow-up of a patient who underwent surgery at age three weeks to correct a right pulmonary artery from the aorta.

Adult↗
PubMed1991

Leiomyosarcoma of the left atrium: case report and review of the literature.

Leiomyosarcomas are extremely rare primary cardiac tumours. A 46-year-old woman presenting with symptoms and signs of rapidly progressive left ventricular failure and apparent systemic lupus erythematosus was subsequently found to have a grade III/III left atrial leiomyosarcoma which was confirmed surgically. Pathology showed a cellular neoplasm arranged in fascicles with multinucleated giant cells, with areas of high grade sarcomatous change. The patient died seven months postoperatively with intractable heart failure. At autopsy, tumour infiltrated the pericardium, both atria and the right ventricle, with invasion of the diaphragm and posterior mediastinum. The current world literature is reviewed with respect to this rare and often misdiagnosed tumour.

Female↗
PubMed1978

Technetium-99m stannous pyrophosphate scintigraphy in patients with calcification within the cardiac silhouette.

Technetium-99m stannous pyrophosphate scintiscanning was performed in 22 patients with radiographically detected calcification within the cardiac silhouette. All but one of these scintigrams showed a localised area of increased activity similar to that ordinarily seen in acute myocardial infarction. Scintiscans in 3 patients after removal of the calcified aortic valve reverted to negative. It was concluded that this technique for acute infarct detection may yield false positive results in the presence of cardiac calcification.

Calcinosis↗
PubMed1978

Cardiac manifestations of malignant hyperthermia susceptibility.

Malignant hyperthermia is a disease resulting from defective cellular membranes, usually presenting as drug-induced pyrexic crises. We describe four patients with life threatening ventricular arrhythmias or chest pain in the absence of pyrexic crises. Three presented with life threatening arrhythmias and the fourth with severe atypical chest pain. Two patients had a family history of multiple sudden deaths. Resting CKs were elevated in three patients while CK-MB was elevated in one. Resting ECGs were abnormal in three. Three patients had recurrent ventricular tachycardia, two had recurrent ventricular fibrillation and multiple cardiac arrests. Cardiac catheterization showed abnormal left ventricular wall motion in two and minimal mitral valve prolapse in one while all had normal coronary arteries. Thallium-201 myocardial imaging demonstrated large perfusion defects in the patient with electrocardiographic Q waves and normal coronary arteries. Myocardial involvement has been demonstrated by clinical, electrocardiographic, hemodynamic, angiographic and myocardial imaging abnormalities. Malignant arrhythmias occurred in these patients in the absence of pyrexic crises or drug admininstration. Abnormal calcium release in the myocardium, as documented in skeletal muscle membranes, may be a unifying concept for the various manifestations described.

Adult↗
PubMed1977

Basement membranes of small cardiac vessels in patients with diabetes and myxoedema: preliminary observations.

Ultrastructural observations on the basement membranes of small vessels from hearts of patients with diabetes, myxoedema and neither disease collected post mortem, reveal that the membranes of those with diabetes are nearly three times as thick as those in normal hearts (P less than 0.001), while in patients with myxoedema they are twice normal thickness (P less than 0.001). A significant difference was demonstrated between the thickness of the basement membrane in patients with diabetes and those with myxoedema (P less than 0.001).

Adult↗
PubMed1976

Prinzmetal's angina with coronary artery spasm. Angiographic, pharmacologic, metabolic and radionuclide perfusion studies.

We studied the effects of coronary artery spasm on perfusion of the microvasculature in a patient with Prinzmetal's angina. Intracoronary injections of 99mTc and 131I-labelled macroaggregated human serum albumin were performed (1) at rest, (2) during spontaneous angina, (3) after the administration of nitroglycerin and (4) during pacing-induced spasm and the resultant scans compared. The resting scan was normal. Pain and spasm were associated with a perfusion defect that was localized to the anterior and inferior walls of the left ventricle. The localization of the perfusion defect corresponded with angiographically demonstrated spasm involving left anterior descending and distal circumflex coronary arteries. A subsequent myocardial infarction was localized by 43K scanning to the same perfusion area. Metabolic and parasympathetic stimulation studies were performed but were inconclusive. The patient's recurrent pains were ultimately controlled with large oral doses of isosorbide dinitrate.

Angina Pectoris↗
PubMed1976

Evolving indications for preoperative intraaortic balloon pump assistance.

Over a two-year period about 1,000 operations were performed with cardiopulmonary bypass. Intraaortic balloon pump assistance (IABP) was employed on 150 occasions, and a review of these has permitted clarification of the indications for its use. Sixty patients had IABP for carcinogenic shock either after infarction or cardiotomy, and 37 (62%) survived. Preoperative IABP in 90 high-risk patients resulted in survival for 79 (88%). The indications for prophylactic IABP included: (1) relief of severe pain, which occurred in 42 patients with acute coronary insufficiency, (2) improvement in the coronary perfusion pressure, which was accomplished in 20 patients with significant left main coronary artery occlusion or its equivalent, and (3) protection of left ventricular function, which war carried out in 28 patients with an LV ejection fraction of less than 0.40. The significance of the preoperative endocardial viability ratio (EVR) in relation to prophylactic IABP was also assessed: an EVR below 0.70 appears to be an indication for preoperative IABP.

Assisted Circulation↗