Thyroid hormones have a considerable influence on cardiac function and structure. There are direct and indirect effects of thyroid hormone on the cardiovascular system, which are prominent in both hypothyroidism and hyperthyroidism. In this review, we discuss how thyroid dysfunction impacts cardiovascular pathophysiology and the underlying molecular mechanisms.
The incidence of right-sided infective endocarditis (RSIE) is steadily increasing and it has been reported to be associated with high risk of embolic events (EE). Aim of our study was to identify the clinical characteristics of patients with RSIE complicated by PE. Indeed, the identification of patients at high risk of significant PE who will benefit from a more aggressive therapeutic strategy may improve the prognosis. From January 2015 to September 2020, 176 patients (Pts) in 6 centers were found to have definite RSIE complicated by PE. Advanced imaging for PE including computed tomography pulmonary angiography (CTPA) was performed in 28 pts (16%) who represent our study group (24 male, mean age 50.6 ±18.29 years). They all underwent transesophageal echocardiography (TEE), in 12 cases (43%) also three-dimensional (3D) TEE, and 27 patients (99%) had both TEE and transthoracic echocardiography (TTE). A total of 53 vegetations (V) were detected. In 18 pts (64%) two or more vegetations were found. Native tricuspid valve was the most frequently involved valve (38 V, 71.7%), followed by catheter (5 V, 9.4%), tricuspid valve prosthesis (4 V, 7.5%), chordae and papillary muscle (2 V, 3.8%) and one vegetation (9%) in each of the following: pulmonic valve, inferior vena cava, eustachian valve, and right atrium. The most common location for vegetations was the anterior leaflet of the tricuspid valve (19 V, 35.8 %) followed by the posterior leaflet (11 V, 20.8%). The most common vegetations morphology was raceme-like shaped (35.8%). Staphylococcus aureus (S. aureus) was the most common causative pathogen (14 pts, 50%). The incidence of PE was very high in patients with vegetation length above 1.5 cm (median 17.6±6.5 mm by TEE). Our results suggest that a routine CTPA should be advised in the presence of vegetations larger than 1.5 cm and with S. aureus infection. This behavior would identify patients at high risk of PE who will benefit from a more aggressive therapeutic strategy, leading to an improvement in the prognosis. Further prospective studies are required to better confirm our hypothesis.
Funding Acknowledgements Type of funding sources: None. Mucopolysaccharidosis (MPS) is a rare genetic lysosomal storage disorder with a wide variability of phenotype. A systematic descriptive study dealing with the echocardiographic (E) features of valvular involvement and their evolution over the time in adolescent and adult patients, whose number is growing up in adult echocardiographic laboratory, is lacking in the medical literature. Purpose To detect the E features of valvular involvement and their evolution in adolescent and adult patients. Method Study design: observational descriptive retrospective. Study group: 142 E studies in 17 adolescent and adult patients with diagnosis of MPS from 2001 until 2020. Mitral (M), aortic (A), tricuspid (T) valves (V) E features (thickness, mobility, calcification, and function), their evolution over the time and the behaviour with Enzyme replacement therapy (ERT) or bone marrow transplant (BMT) were assessed. Result 52% male, mean age 21 yrs ranges 16 to 48 yrs. 5% of the patient had MPS type I, 11% MPS type II, 29% MPS type IV, and 52% MPS type VI. 70% received ERT and 11% BMT. In the severe case all the valves were involved (panel A, B, C; white arrows point to valve leaflets; yellow arrows MV apparatus) the whole MV apparatus was involved since the earlier stage and in the latest stage the calcification was massive (panel C). The predominant valvular dysfunction was the regurgitation followed by mixed disorder while the most frequent severe lesion was the stenosis; the echocardiographic pattern differs from the classical hockey stick appearance of the early phase of rheumatic MV and the thickening is different from the myxomatous MV for the reduced mobility and the presence of calcification. The reduced mobility of the TV (panel C) also differs from the Loeffler syndrome because of the restriction of the leaflets and the association with thickening and calcification. Under treatment, the MV thickening was found to have a slow progression of less than 1 mm yearly in 61% cases. Conclusion Our results showed that all the valves are affected mainly the MV; the echocardiographic pattern of MPS, different from other valvular diseases of adolescent and adult age, can help in avoiding misdiagnose. Our observations also suggest that the cardiac involvement show slow rate of progression after the initiation of the therapy. Further studies are required to confirm our results. Type of valve % of valve thickness % of reduced mobility % calcification diffuse % valve lesion Mitral valve 88% 65% 47% 75% Aortic valve 76% 23% 41% 57% Tricuspid valve 82% 47% 17% 52% Abstract Figure. Echocardiographic features
Mucopolysaccharidoses, a rare inherited disorder of lysosomal storage, account for less than 0.1% of all genetic diseases. The penetrance is highly variable and clinically it varies from severe fetal-neonatal forms to attenuated diseases diagnosed in adult individuals. The majority of the patients have been reported to show cardiac abnormalities since pediatric age, however, there is a minority of patients with attenuated disease diagnosed in the adolescent and adult age. The haematopoietic stem cell transplantation and enzyme replacement therapy are the current therapies for these disorders. Thanks to these treatments, Mucopolysaccharidoses patients live longer than in the past. Even though enzyme replacement therapy has been demonstrated to reduce left ventricular mass in patients with cardiomyopathy, the efficacy on valve abnormalities has not been clearly demonstrate yet. Furthermore, thanks to the current therapy, to better understanding and to the advent on new technologies, an increasing number of adolescent and adult patients diagnosed with MPS are followed up in the adult echocardiographic laboratory. Indeed, a systematic descriptive study describing the echocardiographic features of valvular involvement and their evolution in adolescent and adult patients lacks of medical literature and this was the aim of our investigation. Our results showed that all the valves are affected, mainly the mitral valve with a higher prevalence compared to the pediatric age. The echocardiographic features of MPS differs from other valvular disease of adolescent and adult age, and knowing them can avoid misdiagnosis. Our observations also suggest that the progression of cardiac involvement slows after the initiation of the therapy in our group of age. Further studies on larger population are required to confirm our results.
Funding Acknowledgements Type of funding sources: None. Introduction Valvular aneurysm (VA) is a rare disease whose etiology most frequently includes infective endocarditis but also connective tissue or degenerative myxomatous diseases or traumatism related to a remote surgical procedure. Purpose: Our study aims to describe clinical and echocardiographic features of VA. Methods: 12 years retrospective observational study. Inclusion criteria: patients (pts) found to have a VA by echocardiography (E). A VA was defined as a saccular bulging or a cyst-like outpouching of a valve leaflet that expands and collapses during systole or diastole. The ability of different E techniques in imaging and sizing the valve aneurysm and clinical data were collected. Results: In a 12 years observational period, 12 pts (7 male, 5 female) with a mean age of 41 years ± 16.6 were found to have VA as diagnosed by two experienced readers. In 10 pts there was a diagnosis of IE according to the ESC, AHA criteria. In two pts IE work up was negative. In the patient with definite IE, blood cultures were reported as positive in 7/10 pts (staphylococci 4 pts, Pseudomonas 2 pts, clostridium difficile 1) and 9 pts out of 10 pts underwent surgery and 1 died; all of them had valve replacement except one had mitral valve (MV) repair. Severe regurgitation was found in 9 cases. Associated IE features were: perforation (11 pts), abscess (3pts), vegetation (7pts), fistula (1pt), and embolism (6 pts). Echo features are reported in table 1. Transthoracic E was able to image the VA in only 3/12 pts. Discussion: The spectrum of the VA in our cohort is very unusual. We reported a VA in a bioprosthesis MV not IE related and never described, one case of valvular aneurysm in a bicuspid aortic valve, and 2 cases in the posterior mitral leaflet (PML). The etiology of VA was related to IE as the leading cause (ten in our cohort). In the two pts where no IE was diagnosed, the possible pathogenesis in one patient may have been related to a remote surgical procedure (left atrial dissection) and in the second patient to degenerative phenomena of the MV bioprosthesis. Being the imaging uncommon, it is important to not misinterpret the VA features with large vegetations, cystic lesions, and abscess. Conclusion: In our series, both the typical spectrum of the disease and less common presentations have been found. We reported one of the largest series of VA with never described unusual presentation. 2-dimensional (D) transesophageal E (TEE) was the key E modalities in the diagnosis integrated by 3D TEE allowing an anatomical imaging useful in surgical decision planning. Almost all cases were associated with perforation and severe regurgitation and a high incidence of embolism. All the cases with IE required surgery except one that died while medical therapy and follow up in the other etiologies . The unusual echocardiographic features have to be as early as possible detected in order to let the patient have the best therapeutical interventions. Abstract Table 1: Echocardiographic features Abstract Figure. Valvular aneurysm imaging
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