huge coronary artery aneurysm in osteogenesis imperfecta: a case report

Osteogenesis imperfecta [OI] as an inherited connective tissue disorder can affect all tissues that contains type I collagen. Well-known cardiac complications of this disease such as aortic root dilatation, aortic regurgitation and mitral valve prolapse have been rarely reported in the literature. Coronary artery aneurysm is a rare cardiac complication in OI, as reported in a 19 year old female presenting with myocardial infarction and hypotension

Dilated cardiomyopathy in two patients with xeroderma pigmentosum disease: a case report

Xerodermapigmentosum [XP], is an autosomal recessivegenetic disorder of DNA repair in which the ability to repair damage caused by ultraviolet [UV] light is deficient. The oxidative stress caused by decline catalase activity as an antioxidant enzyme, has been illustrated in these patients. This is the first case report of dilated cardiomyopathy in two patients with XP, A 26 year old girl and her younger brother. Laboratory studies demonstrated severe vitamin D deficiency in both of them. Cardiac dysfunction in the presented cases with XP might be caused by vitamin D deficiency. But this question still remains: whether chronic oxidative stress can involve the heart and can be a predisposing factor or even an underlying factor for dilated cardiomyopathy in XP, or not. More studies are needed for demonstrating this hypothesis

Electrocardiographic findings and serum troponin I in carbon monoxide poisoned patients

Carbon monoxide [CO] poisoning, though with different sources, is one of the most deadly emergencies in all countries. CO can threaten men's life by several paths especially cardiac complications, which can mimic other cardiac problems such as myocardial infarction. The objective of this study was to determine ECG findings and serum troponin I levels in CO poisoned patients. In this analytical cross-sectional study, 63 CO poisoning patients were consecutively included from hospital's emergency departments. CO content was measured by a CO-oximeter and an electrocardiography was taken first thing on admission. Arterial blood gas [ABG], troponin I and other data was collected afterwards. Data were divided by age groups [adults and children] and gender. CO content was significantly higher only in subjects with normal T wave compared to patients with inverted T wave in their initial ECG [P=0.016]. No other significant difference was noticed. None of the ABG findings correlated significantly with CO content. Also no significant correlation was found with CO content after stratification by gender and age groups, but pH in children [r=-0.484, P=0.026]. CO content was significantly higher in adults [P=0.023], but other ABG data were not significantly different. Only 3 patients had elevated troponin I. Receiver operating characteristic [ROC] analysis showed no significant cutoff points in CO content for ECG changes. No significant specific change in electrocardiograms [ECG] could contribute carboxyhemoglobin content in carbon monoxide poisoned patients. In addition, no specific difference was found between adults and pediatric subjects' ECGs. All other findings seemed to be accidental

Pulsatility index in aortic coarctation: a possible way to evaluate factors affecting stenting outcome

The pulsatility index [PI] shows continuous blood flow to the end organs and is a significant factor believed to decrease in aortic coarctation. Correction of this factor is of great importance in the treatment ofstenotic lesions of the aorta. However, there are minimal data regarding the trend of changes in the PI after stent implantation. Furthermore, the association between the PI and other echocardiographic indices in patients undergoing stent implantation is unclear. This study was designed to evaluate changes in the PI following stenting and its correlation with other echocardiographic indices. Twenty-three patients with a diagnosis of aortic coarctation consecutively underwent two-dimensional and Doppler echocardiographic imaging modalities twice [before and after stenting]. The patients were divided into two groups based on the percentage of increase in the PI after stenting [ 50%]. The relation between the post-stenting PI and the baseline echocardiographic indices was assessed. The PI was increased from 0.89 [SD = 0.30] to 1.75 [SD = 0.51] after stenting [p value < 0.001]. Baseline diastolic/systolic velocity [D/S velocity] ratio of the abdominal aorta [p value = 0.013], mean velocity [p value = 0.033], and peak gradient of the descending aorta [p value = 0.033] were significantly higher in the patients with >/= 50% increase in the PI after stenting. Our findings showed that elevation in the PI after stenting was a predictable criterion in patients with aortic coarctation: it was predicted by some baseline clinical and echocardiographic indices. Baseline D/S ratio velocity of the abdominal aorta, mean velocity and peak gradient of the descending aorta, and baseline systolic blood pressure were the statistically significant indices to predict >/= 50% increase in the PI in our patients

aneurysmal interatrial septum with a patent foramen ovale and multiple fenestrations closed by a single occluder device

The patent foramen ovale [PFO] usually is a very small potential opening in the atrial septum. Under the conditions of normal hemodynamics with higher left atrial than right atrial pressures, the septum primum is forced against the foramen by the higher left atrial pressure and there is no actual persistent opening through the foramen. However, with any, even transient, increase in right atrial pressure this flap or "valve" can be pushed away from the septum and forced open. This results in the shunting of blood and anything else in the right atrium from the right atrium to the left atrium. Often the "valve" of the foramen becomes redundant and develops an "aneurysm" of the atrial septum. A large, redundant septum primum can have several additional openings or "fenestrations" in it. The PFO is now can be treated by interventional percutaneous therapy. This case represents a 24-year-old male with an aneurysmal interatrial septum and patent foramen ovale associated with multiple fenestrations. The defects were closed by a single Amplatzer[registered sign] septal occluder

rare electrocardiographic manifestation of a rare form of multiple electrolyte disturbances: hyperparathyroid crisis

The surface electrocardiogram [ECG] has been used as a useful method for detection of metabolic disturbances for a long time. However, it may be difficult to distinguish the exact disturbance when more than one metabolic abnormality exists in a patient simultaneously. Although, [classic] ECG characterizations of common electrolyte disturbances are well described, multiple concurrent electrolyte disturbances may lead to ECG abnormalities that may not be easily detectable. This ECG concerns a 60-year-old male presented with general fatigue, weakness, epigastric pain, anorexia, nausea and extreme hypercalcemia [serum total and ionized calcium levels 20.5 mg/dL and 12.02 mg/dl, respectively], hypokalemia and hypomagnesemia associated with elevated parathyroid hormone [1160 pg/ml] and normal serum vitamin D level [97 ng/ml]. This rare manifestation of primary hyperparathyroidism has been named hyperparathyroid crisis in the literature. Hyperparathyroid crisis is an emergency form of multiple electrolyte abnormalities that manifest as a life-threatening hypercalcemia and simultaneous hypokalemia and hypomagnesemia; these two later are believed to be caused by diuretic effect of calcium on the renal tubules. The unique pattern of ECG in our patient first was misdiagnosed as prominent T waves with prolongation of the QT corrected [QTc] interval, which has been reported several times in patients with hyperparathyroidism crisis, compatible with our patient. But more investigation revealed that, the QTc interval not only is not prolonged, it is shortened as it is expected from the effect of hypercalcemia on electrocardiogram. The exact pattern of the patient's ECG [Figure 1] can be interpreted as it follows: [1] Flattening of the T wave, [2] a prominent U wave, [3] prolongation of the descending limb of the T wave such that it overlapped with the next U wave [4] virtual absence of ST segment and [5] shortening of the QT corrected interval. In conclusion, it should be emphasized when the T and U waves are separated by a very short segment they can mimic the appearance of a prolonged QT interval. However, more investigation can demonstrate the exact electrocardiographic pattern especially in multiple electrolyte disturbances, when [classic] ECG patterns are not expectable

Plasma homocysteine level and coronary artery disease in type 2 diabetic patients without nephropathy

Elevated plasma homocysteine [Hcy] is considered to be a novel risk factor for coronary atherosclerosis. Considering the important role of the kidneys in Hcy clearance, the relation, if any, between Hcy and diabetes mellitus [DM] and coronary artery disease [CAD] in diabetic patients is still unclear. The aim of this study was to investigate whether plasma Hcy is a predictor of CAD in patients with type 2 DM without any evidence ofnephropathy. Among type 2 DM patients without nephropathy [cr 50 percent in at least one coronary artery. The extent of CAD was determined by the number of affected coronary arteries. The mean +/- SD Hcy level was 11.35 +/- 3.7 micro mol/l. No significant difference was observed between the CAD and control groups [11.52 +/- 4.01 micro mol/l vs. 11.02 +/- 3.11 micro mol/l, p=0.440]. CAD was related to body mass index [p=0.044], systolic blood pressure [p=0.027], HDL-c level [p=0.06], serum creatinine [p=0.042], and HdA1c level [p=0.001]. A binary logistic regression analysis found systolic blood pressure e[OR: 0.96, p=0.003], creatinine [OR: 24.76, p=0.013], and Hba1c [Or: 2.41, p=0.017] as independent predictors of the presence of CAD. Predictors of the extent of CAD were history of hypertension and current smoking. In the presence of normal renal function, plasma Hcy level cannot predict either presence or extent of CAD in patients with type 2 DM without nephropathy