Aortic root replacement for aortic root aneurysm with severe aortic regurgitation and incidentally detected left ventricular hyper-trabeculation/noncompaction.

A 54-year-old male with severe aortic regurgitation (AR), aortic root aneurysm, left ventricular hyper-trabeculation/noncompaction (LVHT) and systolic dysfunction with a left ventricular ejection fraction (LVEF) of 52% underwent successful aortic root replacement. Intraoperative video-endoscopy confirmed LVHT. At 3-year follow-up, he remains in an excellent clinical condition and echocardiography shows an improvement of the systolic function, LVHT and LVEF of 66%. Timely surgical correction of severe AR may also lead to improvement of systolic function in a patient with LVHT.

Left Ventricular Noncompaction Detected by Cardiac Magnetic Resonance Screening: A Reexamination of Diagnostic Criteria.

In a previous cross-sectional screening study of 5,169 middle and high school students (mean age, 13.1 ± 1.78 yr) in which we estimated the prevalence of high-risk cardiovascular conditions associated with sudden cardiac death, we incidentally detected by cardiac magnetic resonance (CMR) 959 cases (18.6%) of left ventricular noncompaction (LVNC) that met the Petersen diagnostic criterion (noncompaction:compaction ratio >2.3). Short-axis CMR images were available for 511 of these cases (the Short-Axis Study Set). To determine how many of those cases were truly abnormal, we analyzed the short-axis images in terms of LV structural and functional variables and applied 3 published diagnostic criteria besides the Petersen criterion to our findings. The estimated prevalences were 17.5% based on trabeculated LV mass (Jacquier criterion), 7.4% based on trabeculated LV volume (Choi criterion), and 1.3% based on trabeculated LV mass and distribution (Grothoff criterion). Absent longitudinal clinical outcomes data or accepted diagnostic standards, our analysis of the screening data from the Short-Axis Study Set did not definitively differentiate normal from pathologic cases. However, it does suggest that many of the cases might be normal anatomic variants. It also suggests that cases marked by pathologically excessive LV trabeculation, even if asymptomatic, might involve unsustainable physiologic disadvantages that increase the risk of LV dysfunction, pathologic remodeling, arrhythmias, or mural thrombi. These disadvantages may escape detection, particularly in children developing from prepubescence through adolescence. Longitudinal follow-up of suspected LVNC cases to ascertain their natural history and clinical outcome is warranted.

Pheochromocytoma-Induced Takotsubo Cardiomyopathy.

Pheochromocytoma, a rare catecholamine-secreting tumor, typically manifests itself with paroxysmal hypertension, tachycardia, headache, and diaphoresis. Less often, symptoms related to substantial hemodynamic compromise and cardiogenic shock occur. We report the case of a 66-year-old woman who presented with abdominal pain. Examination revealed a large right adrenal mass, cardiogenic shock, and severe heart failure in the presence of normal coronary arteries. Within days, the patient's hemodynamic status and left ventricular ejection fraction improved markedly. Results of imaging and biochemical tests confirmed the diagnosis of pheochromocytoma-induced takotsubo cardiomyopathy. Medical therapy and right adrenalectomy resolved the patient's heart failure, and she was asymptomatic postoperatively. We recommend awareness of the link between pheochromocytoma and takotsubo cardiomyopathy, and we discuss relevant diagnostic and management principles.

Reversible Cerebral Vasoconstriction Syndrome Associated with Coronary Artery Vasospasm.

Reversible cerebral vasoconstriction syndrome is a rare disorder associated with neurologic symptoms secondary to diffuse cerebral vasospasm. Cardiac involvement in this disease is exceedingly rare. A 50-year-old woman was admitted to our hospital for evaluation of chest pain. During a 3-year period, she had been admitted multiple times because of chest pain and elevated serum cardiac enzymes. Transthoracic echocardiograms showed transient wall-motion abnormalities; however, coronary angiograms revealed no coronary artery disease. At the current admission, she had a thunderclap headache, and cerebral angiograms revealed diffuse cerebral vasoconstriction that improved after verapamil infusion, confirming the diagnosis of reversible cerebral vasoconstriction syndrome. The patient was treated successfully with oral diltiazem and had no recurrence of symptoms. We describe what we think is the first reported case of coronary artery spasm in association with reversible cerebral vasoconstriction syndrome. Future research should be focused on identifying treatment options and defining the mechanisms by which the cerebral and coronary vasculature are affected.

Left Ventricular Noncompaction Cardiomyopathy Presenting with Heart Failure in a 35-Year-Old Man.

Isolated ventricular noncompaction, a rare genetic cardiomyopathy, is thought to be caused by the arrest of normal myocardial morphogenesis. It is characterized by prominent, excessive trabeculation in a ventricular wall segment and deep intertrabecular recesses perfused from the ventricular cavity. The condition can present with heart failure, systematic embolic events, and ventricular arrhythmias. Two-dimensional echocardiography is the typical diagnostic method. We report a case of heart failure in a 35-year-old man who presented with palpitations. Two-dimensional echocardiograms revealed left ventricular noncompaction, which markedly improved after standard heart failure therapy.

Coronary artery calcium score: current status.

The coronary artery calcium score plays an Important role In cardiovascular risk stratification, showing a significant association with the medium- or long-term occurrence of major cardiovascular events. Here, we discuss the following: protocols for the acquisition and quantification of the coronary artery calcium score by multidetector computed tomography; the role of the coronary artery calcium score in coronary risk stratification and its comparison with other clinical scores; its indications, interpretation, and prognosis in asymptomatic patients; and its use in patients who are symptomatic or have diabetes.

O escore de cálcio coronariano tem papel relevante na estratificação de risco cardiovascular, apresentando significativa associação com a ocorrência de eventos cardiovasculares maiores no acompanhamento de médio e longo prazo. São discutidos: os protocolos de aquisição e quantificação por meio da tomografia computadorizada multidetectores; seu papel na estratificação de risco coronariano e relação com os demais escores clínicos; suas indicações, interpretação e prognóstico em pacientes assintomáticos; sua utilização em pacientes sintomáticos e em diabéticos.

Coronary artery calcium score: current status / Escore de cálcio coronariano: estado atual

Abstract The coronary artery calcium score plays an Important role In cardiovascular risk stratification, showing a significant association with the medium- or long-term occurrence of major cardiovascular events. Here, we discuss the following: protocols for the acquisition and quantification of the coronary artery calcium score by multidetector computed tomography; the role of the coronary artery calcium score in coronary risk stratification and its comparison with other clinical scores; its indications, interpretation, and prognosis in asymptomatic patients; and its use in patients who are symptomatic or have diabetes.

Resumo O escore de cálcio coronariano tem papel relevante na estratificação de risco cardiovascular, apresentando significativa associação com a ocorrência de eventos cardiovasculares maiores no acompanhamento de médio e longo prazo. São discutidos: os protocolos de aquisição e quantificação por meio da tomografia computadorizada multidetectores; seu papel na estratificação de risco coronariano e relação com os demais escores clínicos; suas indicações, interpretação e prognóstico em pacientes assintomáticos; sua utilização em pacientes sintomáticos e em diabéticos.

Serum versus Imaging Biomarkers in Friedreich Ataxia to Indicate Left Ventricular Remodeling and Outcomes.

Patients with Friedreich ataxia typically die of cardiomyopathy, marked by myocardial fibrosis and abnormal left ventricular (LV) geometry. We measured procollagen I carboxyterminal propeptide (PICP), a serum biomarker of collagen production, and characterized genotypes, phenotypes, and outcomes in these patients. Twenty-nine patients with Friedreich ataxia (mean age, 34.2 ± 2.2 yr) and 29 healthy subjects (mean age, 32.5 ± 1.1 yr) underwent serum PICP measurements. Patients underwent cardiac magnetic resonance imaging and outcome evaluations at baseline and 12 months. Baseline PICP values were significantly higher in the patients than in the control group (1,048 ± 77 vs 614 ± 23 ng/mL; P <0.001); severity of genetic abnormality did not indicate severity of PICP elevation. Higher PICP levels corresponded to greater LV concentric remodeling only at baseline (r=0.37, P <0.05). Higher baseline PICP strongly indicated subsequent increases in LV end-diastolic volume (r=0.52, P=0.02). The PICP levels did not distinguish between 14 patients with evident myocardial fibrosis identified through positive late gadolinium enhancement and 15 who had no enhancement (1,067 ± 125 vs 1,030 ± 98 ng/mL; P=0.82). At 12 months, cardiac events had occurred in 3 of 14 fibrosis-positive and none of 15 fibrosis-negative patients (P=0.1); their baseline PICP levels were similar. We conclude that PICP, a serum marker of collagen synthesis, is elevated in Friedreich ataxia and indicates baseline abnormal LV geometry and subsequent dilation. Cardiac magnetic resonance and PICP warrant consideration as complementary biomarkers in therapeutic trials of Friedreich ataxia cardiomyopathy.

Takotsubo Cardiomyopathy in a 22-Year-Old Single-Ventricle Patient.

We report a highly unusual case of a 22-year-old woman with single-ventricle anatomy and a history of Fontan palliation who developed takotsubo cardiomyopathy. To our knowledge, takotsubo cardiomyopathy has not previously been described in a patient with single-ventricle anatomy, or more generally in any patient with congenital heart disease. The patient presented at our clinic for urgent examination after a 2-day history of chest pain that had begun upon the death of her dog. Invasive evaluation was refused by the patient; instead, she underwent electrocardiogram-gated coronary computed tomographic angiography, to rule out obstructive coronary disease. A physician who suspects takotsubo cardiomyopathy in a patient should look for new-onset chest pain and dyspnea coupled with these features: immediate onset of segmental akinesia in the midventricular or apical aspects of the heart, elevation of brain natriuretic peptide level 12 to 24 hours after onset of akinesia, elevation of troponin level 24 to 48 hours after onset, and disappearance of the segmental akinesia a few days after onset.

Cardiac magnetic resonance imaging for the investigation of cardiovascular disorders. Part 2: emerging applications.

Cardiac magnetic resonance imaging has emerged as a robust noninvasive technique for the investigation of cardiovascular disorders. The coming-of-age of cardiac magnetic resonance-and especially its widening span of applications-has generated both excitement and uncertainty in regard to its potential clinical use and its role vis-à-vis conventional imaging techniques. The purpose of this evidence-based review is to discuss some of these issues by highlighting the current (Part 1, previously published) and emerging (Part 2) applications of cardiac magnetic resonance. Familiarity with the versatile uses of cardiac magnetic resonance will facilitate its wider clinical acceptance for improving the management of patients with cardiovascular disorders.

Cardiac amyloidosis presenting with prolonged QT interval and recurrent polymorphic ventricular tachycardia.

Cardiac amyloidosis results in severely symptomatic heart failure that has a poor prognosis because of the development of a restrictive cardiomyopathy. The diagnosis of cardiac amyloidosis is often delayed because of nonspecific signs and symptoms. We report the case of a 66-year-old woman who had been diagnosed with sick sinus syndrome and presented 5 months later with a long QT interval and recurrent polymorphic ventricular tachycardia. The diagnosis of cardiac amyloidosis was confirmed upon analysis of endomyocardial biopsy results. The patient was subsequently diagnosed with and treated for underlying plasma cell myeloma and later died of cardiac arrest. This atypical presentation of cardiac amyloidosis underscores the need to consider it in the differential diagnosis of patients who have ventricular arrhythmias. To our knowledge, the combination of long QT interval and polymorphic ventricular tachycardia has not been previously reported in association with amyloid heart disease.

Inverted Takotsubo cardiomyopathy and the fundamental diagnostic role of echocardiography.

Takotsubo cardiomyopathy is characterized by the development of transient focal wall-motion abnormalities that involve the apical and midventricular segments, in the absence of obstructive coronary artery disease. A variant, inverted takotsubo cardiomyopathy, was described in 2010. We report 3 cases in which each patient's transthoracic echocardiogram revealed the characteristic basal and midventricular segmental akinesis of this variant. This pattern is not associated with coronary artery distribution, and it therefore can be differentiated from coronary artery disease with the use of echocardiography, by evaluating the distribution and temporal changes of akinetic areas.

Isolated left ventricular noncompaction in a 90-year-old man.

Isolated ventricular myocardial noncompaction is a cardiomyopathy that is being diagnosed more frequently in patients of all ages because of increased awareness and improvements in imaging methods. It is an extremely rare cause of heart failure in nonagenarians. We describe the case of a man who presented with heart failure for the first time at 90 years of age. The diagnosis was isolated left ventricular noncompaction. Transthoracic echocardiography showed a trabeculated, sponge-like appearance of the left ventricular apical and inferolateral segments. After medical management, the patient was asymptomatic at the 3-month follow-up examination. Knowledge of ventricular noncompaction is increasing within the cardiology community. Patients who have isolated noncompaction with a limited number of involved ventricular segments can live beyond normal life expectancy without developing heart failure. In addition to discussing our patient's case, we briefly review the relevant medical literature.

Peripartum cardiomyopathy: a review.

Peripartum cardiomyopathy is idiopathic heart failure occurring in the absence of any determinable heart disease during the last month of pregnancy or the first 5 months postpartum. The incidence varies worldwide but is high in developing nations; the cause of the disease might be a combination of environmental and genetic factors. Diagnostic echocardiographic criteria include left ventricular ejection fraction <0.45 or M-mode fractional shortening <30% (or both) and end-diastolic dimension >2.7 cm/m(2). Electrocardiography, magnetic resonance imaging, endomyocardial biopsy, and cardiac catheterization aid in the diagnosis and management of peripartum cardiomyopathy. Cardiac protein assays can also be useful, as suggested by reports of high levels of NT-proBNP, cardiac troponin, tumor necrosis factor-α, interleukin-6, interferon-γ, and C-reactive protein in peripartum cardiomyopathy. The prevalence of mutations associated with familial dilated-cardiomyopathy genes in patients with peripartum cardiomyopathy suggests an overlap in the clinical spectrum of these 2 diseases.Treatment for peripartum cardiomyopathy includes conventional pharmacologic heart-failure therapies-principally diuretics, angiotensin-converting enzyme inhibitors, vasodilators, digoxin, ß-blockers, anticoagulants, and peripartum cardiomyopathy-targeted therapies. Therapeutic decisions are influenced by drug-safety profiles during pregnancy and lactation. Mechanical support and transplantation might be necessary in severe cases. Targeted therapies (such as intravenous immunoglobulin, pentoxifylline, and bromocriptine) have shown promise in small trials but require further evaluation. Fortunately, despite a mortality rate of up to 10% and a high risk of relapse in subsequent pregnancies, many patients with peripartum cardiomyopathy recover within 3 to 6 months of disease onset.

Hypertension, tachycardia, and reversible cardiomyopathy temporally associated with milnacipran use.

Elevated catecholamine levels are a well-recognized cause of various types of cardiomyopathy. Causes of catecholamine elevation include tumors, toxins, drugs, emotional stress, and sepsis. Milnacipran is a dual and equipotent inhibitor of norepinephrine and serotonin uptake. It is frequently prescribed as therapy for fibromyalgia, and the drug has a good safety profile. Herein, we report the case of a 42-year-old woman with undefined connective-tissue disease and fibromyalgia who developed a severe and reversible cardiomyopathy while taking recommended doses of milnacipran. The cardiomyopathy was associated with a hyperadrenergic state manifested by tachycardia, hypertension, and elevated plasma catecholamine levels. The discontinuation of milnacipran and the initiation of anti-failure therapy resulted in complete resolution of the cardiomyopathy in 6 months. To our knowledge, this is the first report of milnacipran as a possible cause of catecholamine-induced cardiomyopathy.

Cardiac sarcoidosis with a rare initial manifestation: sustained monomorphic ventricular tachycardia.

Sarcoidosis is a multisystem disease of unknown cause. Life-threatening complications or sudden death can occur when the disease involves the heart. Because cardiac sarcoidosis has diverse clinical presentations, its diagnosis can be a major challenge for clinicians. It is very rare for the initial manifestation of cardiac sarcoidosis to be sustained ventricular tachycardia, especially in a patient with no prior symptoms or history of the disease. Herein, we discuss the case of a 41-year-old black man who presented with nausea, vomiting, and palpitations on the day after he had consumed alcoholic beverages heavily. Electrocardiographic examination revealed sustained monomorphic ventricular tachycardia. An automatic implantable cardioverter-defibrillator corrected the patient's abnormal heart rhythm, and therapy with steroids and ß-blockers resolved his symptoms. We describe the process that led to the diagnosis of cardiac sarcoidosis in this patient.

Echocardiographic myocardial imaging reveals segmental cardiomyopathy in Churg-Strauss syndrome.

Cardiovascular involvement is the leading cause of morbidity and death in Churg-Strauss syndrome. Herein, we describe the case of a 47-year-old man with Churg-Strauss syndrome, in whom the use of novel echocardiographic techniques revealed segmental cardiomyopathy. Tissue Doppler and speckle-tracking imaging showed that both longitudinal and radial strain were impaired at the septal level and that the impairment of circumferential strain affected left ventricular torsion. Our case shows that advanced echocardiography with myocardial strain imaging in multiple vectors can identify systolic-diastolic abnormalities in a patient with myocardial infiltration and a normal left ventricular ejection fraction.

Diverticular and aneurysmal structures of the left ventricle in adults: report of a case within the context of a literature review.

Left ventricular outpouchings are increasingly detected on cardiovascular imaging. Herein, we describe the case of a 45-year-old man who underwent noncardiac preoperative imaging and was found to have an asymptomatic left ventricular outpouching. The patient underwent successful surgical repair of the structure. When left ventricular outpouchings are detected, the main differential diagnoses are pseudoaneurysm, aneurysm, and diverticulum. The outcomes for these conditions differ substantially, and accurate diagnosis can be crucial in making clinical decisions. We review the relevant medical literature, outline the natural history of these left ventricular abnormalities, and discuss options in regard to their management.

Dual atrioventricular-nodal physiology, elicited by pacing and leading to a reversible cardiomyopathy.

Atrioventricular nodal re-entry tachycardia is the most common form of regular paroxysmal tachycardia in the adult population. This tachycardia is a re-entrant rhythm that uses the anatomic location of the atrioventricular node and its surrounding perinodal atrial tissue. The simplest concept regarding the atrioventricular nodal physiology that allows re-entry is founded upon the postulated existence of 2 atrioventricular nodal pathways with different conduction velocities and refractory periods. Herein, we present the case of a 64-year-old man who had a history of paroxysmal atrial fibrillation; he had a permanent pacemaker for sick-sinus syndrome. He developed a tachycardia-induced cardiomyopathy with a perpetual dual response to the pacemaker stimulus. The tachycardia displayed characteristic dual atrioventricular-nodal physiology that was suppressed by amiodarone therapy, leading to a reversal of the cardiomyopathy. We discuss the mechanisms that surround such phenomena.