Cardiogenic shock with basal transient left ventricular ballooning (Takotsubo-like cardiomyopathy) as first presentation of pheochromocytoma.

Pheochromocytoma is a rare tumor that produces a distant effect by secretion of catecholamines. This tumor usually presents with hypertension and palpitations but it may also cause cardiogenic shock because of catecholamine-induced myocardial dysfunction. We describe a rare case of Takotsubo-like cardiomyopathy as first manifestation of pheochromocytoma with an unusual onset characterized by severe hypotension and transient basal left ventricular ballooning ('inverted' Takotsubo-like cardiomyopathy).

D-2-Hydroxyglutaric aciduria in a patient with a severe clinical phenotype and unusual MRI findings.

We report an infant with intermittent urinary excretion of D-2-hydroxyglutaric (D-2-OHG) acid who died at the age of 10 months from cardiogenic shock due to cardiomyopathy. High urinary concentrations of D-2-OHG and succinic acid, as well as increased levels of lactic acid were detected on three different occasions, whereas a normal urinary profile of organic acids was found on one occasion. The clinical findings of our patient consisted of generalized hypotonia, irritability, developmental delay, generalized tonic seizures, lethargy, cardiomyopathy, and respiratory distress. Cerebral MRI revealed bilateral lesions in the substantia nigra, the periaqueductal area, the medial part of the thalamus, the hypothalamus, the caudate nucleus, putamen and globus pallidus. This pattern is suggestive of a mitochondriopathy. However, respiratory chain enzyme activities were normal in fibroblasts. Exogenous supplementation of D-2-OHG acid strongly inhibited cytochrome-c oxidase activity in fibroblasts from the patient and from normal controls in vitro. The results suggest that our patient has an unusual form of D-2-hydroxyglutaric aciduria (D-2-OHGA), different from the patients published so far, and that the increase of lactic acid and some citric acid cycle intermediates encountered in some patients with D-2-OHGA may be due to a functional defect of the respiratory chain caused by D-2-OHG acid.

Use of dopamine and prazosin combined in the treatment of cardiogenic shock.

Seventeen patients who developed cardiogenic shock after acute myocardial infarction were treated with intravenously infused dopamine. In eight of these patients a stable blood pressure was attained, but oliguria or anuria persisted, and oral treatment with prazosin was instituted. Diuresis occurred in seven of these patients, but was followed by transient hypotension associated with a rapid rise in plasma prazosin levels in three. Four patients left hospital, and there were two long-term survivors. Prazosin may be a useful adjunct to dopamine in the treatment of cardiogenic shock.

Urinary excretion of kallikrein following myocardial infarction.

Kinin has been hypothesized to be involved in the mechanism of the procordialgia, collapse, and shock in myocardial infarction. In spontaneous and experimental animal infarction, the long-lasting lowering of plasma kininogen is perhaps the expression of kinin release from the plasma precursor. More recently, a durable reduction of plasma prekallikrein and of the plasma inhibitor of kallikrein, both evaluated with the kaolin contact method, has been demonstrated to support the implication of the kinin system in the course of myocardial infarction. In the present study, the dialy urinary excretion of kallikrein, according to the Porcelli and Croxatto method, has been studied in a group of patients with acute myocardial infarction and in a group of control patients, Differences between the two groups have been observed. They consist mainly in strong daily oscillations in the amount of urinary kallikrein excretion during the 24 hour period in the group of patients with myocardial infaction. At this moment, however, it is not possible to give a definite interpretation of these results.