Cases reported "Cardiomyopathy, Dilated"

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1/17. Solitary renal myofibromatosis: an unusual cause of infantile hypertension.

    INTRODUCTION: Renovascular disease accounts for the vast majority of cases of infantile hypertension with complications resulting from umbilical arterial catheterization predominating in the neonatal period and fibrodysplastic lesions of the renal artery predominating outside the neonatal period. We report a previously undescribed cause of renovascular hypertension: solitary renal myofibromatosis. CASE REPORT: A 9-month-old male infant was transported to the intensive care unit at Children's Hospital in Denver, colorado, for evaluation and treatment of a dilated cardiomyopathy and severe systemic hypertension. The child was full-term with no perinatal problems. Specifically, the child never required umbilical arterial catheterization. He was well until 6 months of age when his parents noted poor weight gain. At 9 months of age, he was evaluated at the referral hospital for failure to thrive. On examination he was noted to have a blood pressure of 170/110 mm Hg, but no other abnormalities. A chest radiograph showed cardiomegaly. Laboratory studies demonstrated normal electrolytes, blood urea nitrogen, and creatinine. However, urinalysis demonstrated 4 protein without red blood cells. An echocardiogram showed severe left ventricular dilatation with an ejection fraction of 16%. On admission the child was noted to be cachectic. His vital signs, including blood pressure, were normal for age. The physical examination was unremarkable. serum electrolytes, blood urea nitrogen, and creatinine were normal. Echocardiographic studies suggested a dilated hypertrophic cardiomyopathy. He was started on digoxin and captopril. Subsequently, he demonstrated episodic hypertension ranging from 170/90 to 220/130 mm Hg. A repeat echocardiogram 24 hours after admission demonstrated a purely hypertrophic cardiomyopathy. verapamil and nifedipine were added to the treatment regimen in an effort to better control the blood pressure without success. urine and blood for catecholamines and plasma renin activity, respectively, were sent and treatment with phentolamine instituted because of a possible pheochromocytoma. A spiral abdominal computerized tomographic scan revealed a markedly abnormal right kidney with linear streaky areas of calcification around the hilum and also an area of nonenhancement in the posterior upper pole. The adrenals and the left kidney were normal. Doppler ultrasound revealed a decrease in right renal arterial flow. The urinary catecholamines were normal and surgery was scheduled after the blood pressure was brought under control by medical treatment. At surgery, tumorous tissue and thrombosis of the renal artery were found in the right upper pole. A right nephrectomy was performed. Pathologic examination of the kidney showed the presence of a diffuse spindle cell proliferation in the interstitium of the kidney. The angiogenic/angiocentric character of the proliferation was demonstrated in several large renal vessels. The lumen of most vessels was narrowed and some vessels were totally occluded with recanalization and dystrophic calcifications observed. Immunostaining of the tumor demonstrated strong desmin and vimentin positivity and minimal actin positivity in the spindle cells. Mitotic activity was not noted in the spindle cell process. These pathologic changes were consistent with a diagnosis of infantile myofibromatosis (IM). The child's preoperative plasma renin activity was 50 712 ng/dL/h (reference range, 235-3700 ng/dL/h). DISCUSSION: The causes of systemic hypertension in infancy are many although renal causes are by far the most common. Renal arterial stenosis or thrombosis accounts for 10% to 24% of cases of infantile hypertension. renal artery thrombosis is usually a consequence of umbilical arterial catheterization, which can also lead to embolization of the renal artery. renal artery stenosis may result from fibrodysplastic lesions (74%), abdominal aortitis (9%), a complication of renal transplantation (5%), and ren
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2/17. Percutaneous revascularization modalities in heart transplant recipients.

    Accelerated allograft vasculopathy significantly limits the survival of heart transplant recipients. The prevalence of allograft coronary artery disease is as high as 18% by 1 year and 50% by 5 years following heart transplant. heart failure and sudden cardiac death are the two most common clinical presentations. In heart transplant recipients with severe, discrete focal allograft vascular disease, percutaneous balloon angioplasty is a viable palliative option. However, its application is limited by a significant restenosis rate and progression of allograft disease in nontreated segments. Diffuse disease with tapering of vessels may be approached by debulking devices. Emerging revascularization modalities for focal stenoses and some of the diffuse tapering vessels include coronary stents, rotational atherectomy, various wavelength lasers, and, to a lesser extent, directional atherectomy. Conceivably, stents will reduce restenosis rates related to focal, discrete plaques; yet it is unknown whether they will be efficacious in short- and long-term treatment of diffusely diseased segments affected by allograft disease. Accurate assessment of clinical outcomes and long-term evaluation is imperative prior to acceptance of these devices as fundamental interventional tools for treatment of allograft coronary artery disease.
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3/17. Orthotopic heart transplantation with concurrent aortic valve replacement and coronary artery bypass grafting.

    We report a case of successful orthotopic heart transplantation of a donor heart with normal ventricular function, 2-vessel coronary artery disease, and a bicuspid aortic valve, which required concurrent aortic valve replacement and coronary artery bypass grafting. In confronting the disparities in demand and supply, we must consider the so-called marginally acceptable heart for either critically ill recipients or those who may be disadvantaged on the waiting list.
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4/17. Diffuse and severe left ventricular dysfunction induced by epicardial coronary artery spasm.

    Endothelial dysfunction and effectiveness of treatment of calcium antagonists are suggestive of coronary artery spasm as an underlying disorder in dilated cardiomyopathy (DCM). The aim of this study is to determine whether or not the epicardial coronary artery spasm can induce severe cardiac dysfunction like DCM. Thirty-four consecutive patients with angiographically normal coronary arteries and diffuse left ventricular hypokinesis whose causes had been unknown underwent acetylcholine provocation test and left ventricular biopsy. Eight patients were excluded according to the clinical and laboratory data and biopsy findings suggesting myocarditis or other systemic diseases. According to the results of the acetylcholine provocation test, 17 patients were finally diagnosed as having DCM, and nine patients (35% of the study patients), who had acetylcholine-induced diffuse and multivessel coronary spasm, were diagnosed as having DCM-like vasospastic angina pectoris (VSA). Clinical and cardiac catheterization data including hemodynamics and biopsy findings were similar between the two groups except that left ventricular end-systolic volume was significantly greater in DCM than in DCM-like VSA. After the acetylcholine provocation test, DCM patients received both a beta blocker and an angiotensin-converting enzyme inhibitor, and DCM-like VSA patients received antianginal drugs. In echocardiographic findings at predischarge and those after 6-month drug treatment, both DCM-lke VSA and DCM showed significant reduction in end-diastolic and end-systolic diameters and significant increase in fractional shortening and ejection fraction, whereas changes in ejection fraction and fractional shortening were significantly greater in DCM-like VSA than those in DCM. Epicardial coronary artery spasm can induce diffuse and severe left ventricular dysfunction like DCM in VSA. Although antianginal drugs markedly improve left ventricular function of these patients, only the acetylcholine provocation test can identify DCM-like VSA.
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5/17. Combined coronary intervention in heart-transplant patient with rapidly accelerated cardiac allograft vasculopathy.

    A 46-year-old man accepted for heart transplantation due to persistent cardiac failure from dilated cardiomyopathy underwent a transplant in germany on July 13, 1995. The donor heart was suspected of coronary artery disease at explantation, but he could wait no longer because of his rapidly deteriorating hemodynamics. Postoperative coronary angiography revealed 25% stenosis of the left descending artery. He showed several episodes of minimal or moderate rejection postoperatively, and coronary angiography 15 months postoperatively showed rapidly accelerated cardiac allograft vasculopathy demonstrating triple vessel disease with multiple lesions. Percutaneous transluminal coronary angioplasty was successful on 2 coronary vessels, but immediately recurrent stenosis and new lesions involving the left main trunk occurred 6 weeks thereafter. Since he was financially unable to afford a second heart transplantation, quadruple coronary artery bypass grafting was conducted October 25, 1996. A biventricular assist device was used when he could not be weaned from cardiopulmonary bypass. He died of multiple organ failure 3 days after surgery.
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6/17. Aortic valve replacement combined with endoventricular circulatory patch plasty (Dor operation) in a patient with aortic valve stenosis and severe ischemic cardiomyopathy.

    A 58-year-old woman with ischemic cardiomyopathy and aortic valve stenosis, underwent aortic valve replacement and simultaneous endoventricular circulatory patch plasty (Dor operation). She underwent coronary artery bypass grafting for severe triple vessel disease 10 years ago. Recently she started to show severe congestive heart failure. aortic valve stenosis with pressure gradient of 85-mmHg was also found. Coronary bypasses were all patent, but the left ventricle (LV) was severely dilated (LVDd/Ds=71/61 mm) and the ischemic cardiomyopathy was considered as the cause. She successfully underwent aortic valve replacement and endoventricular circulatory patch plasty. The initial postoperative course was complicated with intractable ventricular arrhythmia, but subsequent course was smooth and the patient was discharged with improved symptoms (NYHA Class II). Postoperative catheterization showed decreased left ventricular volume and improved contractility. This case implies the role of LV remodeling procedure in the ischemic cardiomyopathy combined with aortic valve lesion
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7/17. Iterative atrial tachycardia originating from the coronary sinus musculature.

    A case of iterative atrial tachycardia leading to dilated cardiomyopathy is reported. During electrophysiologic study, the tachycardia showed a markedly irregular cycle length associated with changes in atrial activation breakthrough as demonstrated by coronary sinus (CS) recordings and frequently degenerated into self-terminating atrial fibrillation. Left atrial transseptal mapping demonstrated the earliest endocardial atrial activation close to the posterolateral mitral annulus, but this was invariably later than that recorded within the CS, where low-energy radiofrequency applications eliminated the tachycardia. No acute vessel damage was observed at postablation CS angiography. In accordance with previously published experimental data, we hypothesized that the muscular sleeves surrounding the CS might be involved in the genesis of this tachycardia. During 6-month follow-up, the patient remained asymptomatic without tachycardia recurrences and with complete recovery of left ventricular function, confirming the reversible nature of the tachycardia-induced cardiomyopathy.
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8/17. Successful implantation of a coronary sinus lead after stenting of a coronary vein stenosis.

    Dislodgment of the coronary sinus lead was observed in a 79-year-old patient 8 months after implantation of a biventricular pacing system. A severe stenosis in the posterolateral branch, in which the lead was previously positioned, prohibited reinsertion of the lead. Because no other branches with adequate anatomy for lead insertion were available in the targeted area, the stenosis was dilated and stented. Subsequently, the left ventricular lead could be reimplanted in the same vessel.
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9/17. Late bleeding from right internal mammary artery after HeartMate left ventricular assist device implantation.

    Postoperative bleeding is one of the major complications after implantation of left ventricular assist devices. We experienced 5 unusual cases, which had bleeding from the right internal mammary artery between 5 and 69 days after implantation of a HeartMate (Thoratec Corporation, Pleasanton, CA) device. It was evident that the outflow graft had eroded through the vessel. Sudden decreases in device flow, hypotension, bleeding from the driveline or chest tube sites, and a drop in hematocrit were the initial manifestations. Chest roentgenogram and transthoracic echocardiography were effective in identifying hemothorax and cardiac tamponade. Four out of 5 patients survived to heart transplantation and were discharged from the hospital. When identified and treated appropriately, this complication does not impair patient outcome.
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10/17. Ventricular tachycardia induced by biventricular pacing in patient with severe ischemic cardiomyopathy.

    INTRODUCTION: cardiac resynchronization therapy (CRT) is a new alternative which affords symptomatic improvement in two-thirds of patients who exhibit medically refractory congestive heart failure (CHF) as well as significant prolongation of the QRS duration (>135 msec). As more experience with CRT accrues, unexpected complications of this promising therapy may become apparent. Herein, we describe a patient with severe ischemic cardiomyopathy and refractory CHF who developed incessant ventricular tachycardia (VT) after the initiation of biventricular pacing. The patient is a 75-year-old man who suffered an inferior myocardial infarction 6 years before presenting for CRT. He underwent a three-vessel CABG in 1997. Subsequently, episodes of near syncopal sustained VT developed, for which he received a dual chamber ICD. In 2001 he developed refractory CHF and ECG revealed LBBB with a QRS duration of 195 msec. Shortly after the initiation of biventricular pacing, the patient developed multiple episodes of drug resistant monomorphic VT that could be terminated only transiently by ICD therapies. Ultimately, the only intervention, which proved to be effective in eliminating VT episodes, was inactivation of LV pacing. Despite subsequent therapeutic regimen of sotalol, lidocaine, tocainide, and quinidine all subsequent attempts to reactivate LV pacing resulted in prompt VT recurrence. CONCLUSION: This case represents a clear example of CRT induced proarrhythmia, which required inactivation of LV pacing for effective acute management. Such an intervention should be considered in CRT patients who exhibit a notable increase in drug refractory VT episodes.
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