Valvular heart disease in patients with osteogenesis imperfecta.

Osteogenesis imperfecta (OI) or "brittle bone disease" is a rare connective tissue hereditary disorder. The most common clinical presentation of OI is bone fractures. OI also involves extraskeletal structures; however, cardiovascular manifestations are rare. In this report, we describe the cardiovascular anomalies of patients with OI who underwent valve surgery and review the literature on this subject.

Predictors for the development of severe tricuspid regurgitation with anatomically normal valve in patients with atrial fibrillation.

BACKGROUND AND AIMS: Atrial fibrillation (AF) may be a risk factor for severe functional tricuspid valve regurgitation (FTR). We aimed to determine the predictors of severe FTR in patients with AF. METHODS AND RESULTS: From our echocardiographic laboratory database, we searched for and reviewed the medical records of consecutive patients with severe FTR and AF seen at Mayo Clinic in Arizona from 2002 through 2009. Our search identified 42 patients who met all inclusion criteria. These patients (cases) with severe FTR and AF were compared with 38 patients (controls) with AF who had no greater than mild tricuspid regurgitation. Case patients with severe FTR were older than controls (mean, 81 years vs. 76 years; P < 0.001) and more frequently had chronic AF (69% vs 26%; P < 0.001). Mean right atrial volume (86 mL/m(2) vs 46 mL/m(2) ; P < 0.001), right ventricular volume (42 mL ± 33 mL vs 22 mL ±8 mL; P < 0.001) and tricuspid annular diameter (3.6 cm vs 3.0 cm; P < 0.001) were larger in cases than in controls. Patients with severe FTR also had a higher prevalence of right-sided heart failure (69% vs 16%; P < 0.001). After adjusting for age and gender, right atrial and right ventricular volumes were independent predictors for the development of severe FTR in patients with AF (odds ratio, 1.7 [95% CI, 1.3-2.8] for every 10 mL/m(2) increase in right atrial volume; P = 0.0002 and odds ratio, 3.1 [95% CI, 1.5-8.9] for every 10 mL increase in right ventricular volume; P = 0.0002). CONCLUSIONS: Severe FTR occurs in older patients with chronic AF as a result of marked right atrial and right ventricular dilatation; and enlargement of the tricuspid annulus in the absence of pulmonary hypertension. More importantly, severe FTR leads to increased prevalence of right-sided heart failure underscoring the nonbenign nature of chronic AF.

Atypical presentation of takotsubo cardiomyopathy.

Midventricular ballooning syndrome, an atypical variant of takotsubo cardiomyopathy (TCM), is characterized by transient wall motion abnormalities of the midsegment of the left ventricle with apical sparing. In contrast to the typical form of TCM, apical contractility is preserved and may even be hyperkinetic in midventricular TCM. We present a case of atypical TCM in an 86-year-old woman who had chest pain while accompanying her husband in the emergency department as he was evaluated for chest pain.

Anterolateral papillary muscle rupture: an unusual complication of septic coronary embolism.

In most cases, acute mitral valve regurgitation in the setting of infective endocarditis is caused by the destruction of either the mitral valve leaflets or the chordal apparatus. A 54-year-old woman had development of respiratory failure due to pulmonary oedema from severe acute mitral valve regurgitation in the setting of acute bacterial endocarditis. She was found to have a ruptured anterolateral papillary muscle from occlusion of the circumflex artery by embolic vegetations arising from the aortic valve. Although this occurrence is uncommon, an embolic phenomenon resulting in myocardial infarction and subsequent rupture of papillary muscle must be considered as a cause of acute severe mitral valve regurgitation.

Symptomatic pericardial cyst: a case series.

Pericardial cysts are most commonly located at the cardiophrenic angle or, rarely, in the posterior or anterior superior mediastinum. The majority of pericardial cysts are asymptomatic and are found incidentally. Symptomatic pericardial cysts present with dyspnoea, chest pain, or persistent cough. We describe four patients with symptomatic pericardial cysts who were treated with either echocardiographically guided percutaneous aspiration or video-assisted thoracoscopic surgery, or both; thoracotomy; or conservative therapy.

Occurrence of left-sided heart valve involvement before right-sided heart valve involvement in carcinoid heart disease.

Carcinoids are rare neuroendocrine tumours that occur primarily in the gastrointestinal tract. Carcinoid heart disease is characterized by fibrous plaque deposition on the endocardial surface of the cardiac valves and chambers. It affects the right heart valves in 85% of cases and the left heart valves in 15%. We present an unusual case of a patient with metastatic carcinoid heart disease in whom typical carcinoid aortic and mitral valve lesions developed 2 years prior to the development of severe right-sided carcinoid valvular heart disease.

Takotsubo cardiomyopathy in a patient with pulmonary embolism.

Takotsubo cardiomyopathy is an acute cardiac syndrome characterized by transient regional wall motion abnormalities of the left ventricular apex or midventricle. Patients often present with chest pain or dyspnoea, ST-segment elevation, and minor elevations of cardiac enzyme levels. Takotsubo cardiomyopathy has been associated with severe emotional or physical stress such as severe burns, spinal cord injury, subarachnoid haemorrhage, and multiple traumas. We report a case of takotsubo cardiomyopathy in a 79-year-old woman with pulmonary embolism. Although pulmonary embolism has been listed as a potential cause of takotsubo cardiomyopathy, this is the first case reported with this association.

An unusual presentation of hemolytic anemia in a patient with prosthetic mitral valve.

Although rare, periprosthetic valvular regurgitation can cause hemolytic anemia. We present the case of a 63-year-old man who had an unusual presentation of hemolytic anemia due to periprosthetic mitral valve regurgitation (PMVR) in the presence of cold agglutinins. Due to high surgical risk, PMVR was percutaneously closed with three Amplatzer devices under the guidance of three-dimensional transesophageal echocardiography.

Visualization of atrial septal aneurysm and patent foramen ovale by three-dimensional transesophageal echocardiography.

: Transesophageal echocardiography is frequently performed for further evaluation of sources of embolism and better evaluation of atrial septum in patients with cerebral ishemic events. Although two-dimensional transesophageal echocardiography can depict atrial septal aneurysm and patent foramen ovale, the full extent of the patent foramen ovale cannot be easily discerned in some cases. We describe a patient with transient cerebral ischemia where three-dimensional echocardiography provided incremental value when compared to two-dimensional transesophageal echocardiography in the assessment of atrial septal aneurysm and patent foramen ovale.

Predictors of Elevated Cardiac Enzyme Levels in Hospitalized Patients with Atrial Fibrillation and No Known Coronary Artery Disease.

We retrospectively studied the predictive capabilities of elevated cardiac enzyme levels in terms of the prognosis of patients who were hospitalized with atrial fibrillation and who had no known coronary artery disease. Among 321 patients with atrial fibrillation, 60 without known coronary artery disease had their cardiac enzyme concentrations measured during hospitalization and underwent stress testing or cardiac catheterization within 12 months before or after hospitalization. We then compared the clinical and electrocardiographic characteristics of the 20 patients who had elevated cardiac enzyme levels and the 40 patients who had normal levels. Age, sex, and comorbidities did not differ between the groups. In the patients with elevated cardiac enzyme levels, the mean concentrations of troponin T and creatine kinase-MB isoenzymes were 0.08 ± 0.08 ng/mL and 6.49 ± 4.94 ng/mL, respectively. In univariate analyses, only peak heart rate during atrial tachyarrhythmia was predictive of elevated enzyme levels (P <0.0001). Mean heart rate was higher in the elevated-level patients (146 ± 22 vs 117 ± 29 beats/min; P=0.0007). Upon multivariate analysis, heart rate was the only independent predictor of elevated levels. Coronary artery disease was found in only 2 patients who had elevated levels and in one patient who had normal levels (P=0.26). Increased myocardial demand is probably why the presenting heart rate was predictive of elevated cardiac enzyme levels. Most patients with elevated enzyme levels did not have coronary artery disease, and none died of cardiac causes during the 6-month follow-up period. To validate our findings, larger studies are warranted.

Does valvuloarterial impedance impact prognosis after surgery for severe aortic stenosis in the elderly?

BACKGROUND: Valvuloarterial impedance (Zva) was introduced as a prognostic measure in patients with aortic stenosis (AS). However, it is unclear whether Zva has a prognostic impact on survival after surgical aortic valve replacement (AVR) in patients with severe AS with preserved ejection fraction (EF). METHODS: We retrospectively reviewed 929 consecutive patients who had AVR. We investigated 170 elderly patients (age >65 years, mean 76 years) who had AVR secondary to severe AS (mean gradient ≥40 mm Hg; aortic valve area ≤1 cm(2); peak velocity ≥4 m/s). Patients with EF <50%, greater than moderate aortic regurgitation, prior heart surgery and concomitant mitral or tricuspid valve surgery were excluded. Zva was calculated and the patients were divided into two groups; low Zva, Zva <4.3 (n=82) and high Zva, Zva ≥4.3 (n=88). The end point was all-cause of death. Survival curves were calculated according to Kaplan-Meier method. RESULTS: Age, prevalence of hypertension, diabetes, chronic kidney disease (CKD), atrial fibrillation, symptoms, EF, E/e' and concomitant coronary artery bypass graft were not different between the groups. Survival was not different between the groups at 5 years (70% in low Zva and 81% in high Zva; p=0.21) and for the entire follow-up period (p=0.23). Only age was a significant factor in predicting survival by multivariate analyses in Cox proportional hazards model after adjusting for Zva, CKD, atrial fibrillation and hypertension. CONCLUSIONS: Our results suggest that preoperative Zva does not have a prognostic impact on postoperative survival in elderly patients with severe AS with preserved EF. Further investigation is needed to elucidate the controversial results.

Effect of mitral inflow pattern on diagnosis of severe mitral regurgitation in patients with chronic organic mitral regurgitation.

BACKGROUND: To determine sensitivity and specificity of E wave velocity in patients with severe chronic organic mitral regurgitation (MR) and normal left ventricular ejection fraction (EF) and to evaluate prevalence of A wave dominance in patients with severe MR. METHODS: We compared 35 patients with quantified severe, chronic, quantified, organic MR due to flail/prolapsed leaflets who had reparative surgery with 35 age-matched control subjects. EXCLUSION CRITERIA: EF < 60%, atrial fibrillation, and more than mild aortic regurgitation. RESULTS: Mean [standard deviation (SD)] age [70 (8) years vs. 69 (8) years; p = 0.94] and mean (SD) EF [66% (6%) vs. 65% (4%); p = 0.43] were not different between the two groups. Mean (SD) E wave velocity was greater in case patients than control subjects [1.2 (0.3) m/sec vs. 0.7 (0.15) m/sec; p < 0.001]. However, E wave velocity of 1.2 m/sec had a sensitivity of only 57% [95% confidence interval (CI), 41-7 and a specificity of 100% (95% CI, 90-100%) in identifying severe MR. E wave velocity of 0.9 m/sec had a more optimal combined sensitivity (89%; 95% CI, 74-95%) and specificity (86%; 95% CI, 71-94%). A wave dominance was seen in 18% of case patients and 66% of control subjects (p < 0.001). CONCLUSION: E wave velocity of 1.2 m/sec is specific not sensitive for severe organic MR; E wave velocity of 0.9 m/sec has better sensitivity and specificity. A wave dominance pattern alone cannot exclude patients with severe organic MR. Our findings highlight the importance of a comprehensive echocardiographic exam rather than relying on a few Doppler parameters in diagnosing MR.

Predictors of severe tricuspid regurgitation in patients with permanent pacemaker or automatic implantable cardioverter-defibrillator leads.

Patients with permanent pacemaker or automatic implantable cardioverter-defibrillator (AICD) leads have an increased prevalence of tricuspid regurgitation. However, the roles of cardiac rhythm and lead-placement duration in the development of severe tricuspid regurgitation are unclear. We reviewed echocardiographic data on 26 consecutive patients who had severe tricuspid regurgitation after permanent pacemaker or AICD placement; before treatment, they had no organic tricuspid valve disease, pulmonary hypertension, left ventricular dysfunction, or severe tricuspid regurgitation. We compared the results to those of 26 control subjects who had these same devices but no more than mild tricuspid regurgitation. The patients and control subjects were similar in age (mean, 81 ±6 vs 81 ±8 yr; P = 0.83), sex (male, 42% vs 46%; P = 0.78), and left ventricular ejection fraction (0.60 ±0.06 vs 0.58 ± 0.05; P = 0.4). The patients had a higher prevalence of atrial fibrillation (92% vs 65%; P=0.01) and longer median duration of pacemaker or AICD lead placement (49.5 vs 5 mo; P < 0.001). After adjusting for age, sex, and right ventricular systolic pressure by multivariate logistic regression analysis, we found that atrial fibrillation (odds ratio=6.4; P = 0.03) and duration of lead placement (odds ratio=1.5/yr; P = 0.001) were independently associated with severe tricuspid regurgitation. Out study shows that atrial fibrillation and longer durations of lead placement might increase the risk of severe tricuspid regurgitation in patients with permanent pacemakers or AICDs.