The Role of Standard Echocardiographic Parameters in Endomyocardial Biopsy Proven Cardiac Amyloidosis.

Primary light-chain (AL) amyloidosis is a plasma cell dyscrasia associated with the deposition of immunoglobulin-derived amyloid in multiple organs. In the heart, this results in an infiltrative cardiomyopathy, with increased left ventricular wall thickness, normal or decreased left ventricular (LV) cavity size and congestive heart failure. Cardiac involvement is a major determinant of prognosis of AL amyloidosis. We report a patient with cardiac amyloidosis proven by cardiac biopsy, and aim to point out at transthoracic echocardiography as the hallmark of diagnostics. Echocardiography revealed increased LV thickness at 20mm, impaired LV ejection fraction (EF) at 35%, enlarged atria, transmitral deceleration time at 156 ms and increased E/A ratio at 4.25. Early diagnosis and intervention can have a significant impact on the patient's response to treatment, especially when the underlying condition involves a malignancy or infiltrative disorder. Standard transthoracic echocardiography as a noninvasive diagnostic tool is valuable and has a significant role in diagnosis and prognosis.

Pulmonary valve papillary fibroelastoma diagnosed by echocardiography: a case report.

Papillary fibroelastomas (PFEs) are rare cardiac valve tumours with reported incidence of <0.03% according to autopsy studies. Among them, pulmonary valve PFEs are extremely rare. With wider use of echocardiography, they are being increasingly recognized premortem. We describe a case of a 32-year-old Caucasian woman with PFE of pulmonic valve diagnosed by echocardiography. The patient underwent surgery due to high mobility of the tumour and high risk of embolic complications. The surgery was done with complete tumour resection and total preservation of valve function. This case report discusses diagnostics of PFEs, their characteristic echocardiographic and histological features, and possible complications and suggests treatment options in this rare cardiac tumour.

[Varicella zoster myopericarditis in an immunocompetent adult]. / Mioperikarditis izazvan virusom varicella-zoster u imunokompetentnog odraslog bolesnika.

More than 20 viruses have been reported to cause myopericarditis, a rare but potentially dangerous complication. To our best knowledge only a few dozen cases of myopericarditis caused by varicella zoster virus have been reported, most frequently in children, seldom in immunocompetent adults. We report on a case of a myopericarditis caused by varicella zoster virus in a previously healthy young man, with a typical development and a fast and complete recovery. A 27-years-old male was admitted to our hospital with chest pain and signs of acute cardiac injury. He had no medical history of serious previous illnesses. Four weeks earlier, the patient was in contact with a child having chickenpox. Two days prior to admission patient became suddenly febrile up to 40 degrees C along with dry cough, and the following day intense chest pain set in. At the admission we found in the patient diffuse vesiculous exanthema on the whole body and capillitium. Heart beat was rhythmical with no audible murmurs or pericardial friction rub, 100 beats per minute, blood pressure RR 130/90 mm Hg, body temperature 37.4 degrees C, and ST segment elevation in lateral leads along with elevated cardiac markers were found. All parameters of complete blood count were within normal range. Chest X-ray showed somewhat enlarged heart with incipient signs of cardiac decompensation. Echocardiogram was normal, apart from a mild dyskinesis of the apical third of intraventricular septum, with ejection fraction slightly reduced to 50% and no valvular defect. Clinical diagnosis of acute infection with varicella zoster virus was confirmed serologically by a positive ELISA test. Patient received conservative therapy (isosorbide mononitrate, low molecular weight heparin, acetylsalicylic acid and bisoprolol), while he remained hemodinamically, and apart from one non sustained ventricular tachycardia immediately after admission, also rhythmically stable. During his stay in hospital we observed the typical evolution and regression of rash while the levels of cardiac markers normalised, with patient becoming afebrile the third day. Before dismission a control echocardiograph showed improvement of contractile function, ejection fraction improved to 65%, but also signs of mild pericarditis were recorded. Since the patient had no discomfort and was in a very good shape, he was dismissed from hospital with ibuprofen 400 mg twice a day as therapy. Two months later, the patient was readmitted for control. In the mean time he had no discomfort, all laboratory and the physical examination findings were normal, as well as the ECG. Echocardiogram showed normal contractility, systolic and diastolic function. Cardiac stress testing and coronary angiography both ruled out a coronary heart disease. Diagnosis of myocarditis in this case was made based on echocardiogram, anamnestic data and on the typical clinical presentation of an acute varicella zoster virus infection, and was serologically confirmed by ELISA test. Therapy with NSAID was started immediately and because of favorable development of the disease we did not perform myocardial biopsy. During second hospitalization an eventual coronary heart disease was ruled out by coronary angiography. Due to its rarity there are no guidelines regarding therapy of varicella myocarditis, but there is a consensus that these patients should receive intensive care unit. The basis of the therapy are certainly the NSAIDs, but also a combination of acyclovir and hyperimmunoglobulins has been reported which in this mild case was not necessary. With this report we want to point out that varicella zoster virus can cause myopericarditis in immunocompetent adults, which must be taken into consideration in differential diagnosis, and that an early diagnosis and adequate therapy can help achieve a fast and complete recovery.

Role of echocardiography in diagnosis and management of complete papillary muscle rupture caused by myocardial infarction.

AIM: To evaluate the usefulness of echocardiography in the diagnosis of complete rupture of papillary muscle. METHODS: Transthoracic (TTE) and transesophageal echocardiography (TEE) was performed with the ATL 3000 HDI Ultrasound Inc (Bothell, WA, USA) with a 2.5 MHz transducer and 5-7 MHz multiplane phased array transducer. We are reporting about two patients (a 45 and a 51-year old male) with complete ruptures of papillary muscle following acute myocardial infarction (AMI). RESULTS: Both patients were previously treated with fibrinolysis in their local hospitals, 400 and 300 km, respectively, away from our hospital. Massive mitral regurgitation developed in both followed by rapid deterioration of hemodynamic state and severe heart failure, because of which both were transferred by helicopter to the Coronary Care Unit of our clinic. The diagnosis of complete papillary muscle rupture was confirmed in both patients by TTE and TEE. Due to the significant deterioration in their hemodynamic state, vasoactive drugs and intra-aortic balloon pump support were applied. Both patients then underwent mitral valve replacement, accompanied by concomitant coronary artery bypass grafting in one case. CONCLUSION: Transesophageal echocardiography is a more accurate and rapid diagnostic method in patients with mechanical complications of AMI than TTE.

Tetralogy of Fallot with cor triatriatum dexter in an adult patient: a case report.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect with only approximately 3% of uncorrected patients surviving past age 40. In this case report, we present a 48-year-old mentally retarded man suffering from congenital spastic quadriplegia who was diagnosed with a unique combination of symptomatic TOF and cor triatriatum dexter (CTD). Reduced preload because of CTD with spastic quadriplegia that prevented physical exertion is believed to have facilitated this patient's unusually long survival.

Aortic valve endocarditis in a transplanted heart after urethral instrumentation.

Endocarditis represents a rare but life-threatening condition after heart transplantation. Recent American Heart Association guidelines recognize cardiac transplant patients with valvulopathy as high risk for endocarditis, but acknowledge that there were not sufficient data to make a recommendation for prophylaxis. Also, genitourinary procedures were no longer considered a risk factor for endocarditis in the most recent guidelines. We present a patient who acquired aortic valve endocarditis of the intact valve, after multiple urethral instrumentation 2 years after heart transplantation, who was successfully treated by aortic valve replacement and prolonged antibiotic therapy.

A familial cluster of idiopathic dilatation of the right atrium-A two-case report.

We report two cases of idiopathic atrial dilatation in two adult siblings, a brother and a sister. The first patient was a 36-year-old man who was referred to our institution for evaluation of atrial fibrillation and syncopes. Transthoracic echocardiography revealed an enlarged right atrium accompanied by a severe tricuspid regurgitation associated with annular dilatation. The diagnosis of idiopathic atrial enlargement was made after all other lesions known to produce it have been excluded. The patient did not consent to the proposed cardiothoracic surgical treatment so he remained on conservative therapy. On electrocardiography, atrial standstill was noted, resulting in the implantation of a VVI cardiac pacemaker one year later. After an asymptomatic period, the patient suffered sudden death at the age of 40 years. His 45-year-old sister suffering from the same symptoms was also referred for examination, also to be diagnosed with idiopathic atrial dilatation and severe tricuspid regurgitation. Idiopathic dilatation of the right atrium, although a rare disorder, should not be forgotten as differential diagnosis for enlarged right atrium. Transthoracic echocardiography is the most commonly used technique and in our cases it was sufficient for establishing the diagnosis.