A consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology.
AimsHypertrophic cardiomyopathy (HCM) is an important cause of heart failure-related disability over a wide range of ages. Profiles of severe progressive heart failure symptoms and death, or heart transplantation deserve more complete definition within large patient cohorts.
Idiopathic recurrent acute pericarditis (IRAP) is a rare disease of suspected, yet unproved, immune-mediated origin. The finding of serum heart-specific autoantibodies in IRAP would strengthen the autoimmune hypothesis and provide aetiology-specific non-invasive biomarkers.
Objective
To assess frequency of serum anti-heart (AHA), anti-intercalated-disk (AIDA) and non-cardiac-specific autoantibodies and their clinical and instrumental correlates in patients with IRAP.
Patients
40 consecutive patients with IRAP, 25 male, aged 37±16 years, representing a large single-centre cohort collected at a referral centre over a long time period (median 5 years, range 1–22 years). Control groups included patients with non-inflammatory cardiac disease (NICD) (n=160), ischaemic heart failure (n=141) and normal subjects (n=270).
Methods
AHA (organ-specific, cross-reactive 1 and 2 types) and AIDA were detected in serum samples from patients, at last follow-up, and control subjects by indirect immunofluorescence (IIF) on human myocardium and skeletal muscle. Non-cardiac-specific autoantibodies were detected by IIF, and anti-Ro/SSA, anti-La/SSB by ELISA.
Results
The frequencies of cross-reactive 1 AHA and of AIDA were higher (50%; 25%) in IRAP than in NICD (4%; 4%), ischaemic (1%; 2%) or normal subjects (3%; 0%) (p=0.0001). AHA and/or AIDA were found in 67.5% patients with IRAP. Of the non-cardiac-specific antibodies, only antinuclear autoantibodies at titre ≥1/160 were more common in IRAP (5%) versus normal (0.5%, p<0.04). AIDA in IRAP were associated with a higher number of recurrences (p=0.01) and hospitalisations (p=0.0001), high titre (1/80 or higher) AHA with a higher number of recurrences (p=0.02).
Conclusions
The detection of AHA and of AIDA supports the involvement of autoimmunity in the majority of patients with IRAP.
All patients undergoing aortic (AVR, n = 196), mitral (MVR, n = 502), and mitralaortic (MAVR, n = 71) valve replacement with a standard Hancock porcine bioprosthesis (HPB) from 1970 to 1983 were reviewed. A total of 665 patients discharged were followed for 5,099 patient-years with an actuarial survival at 15 years of 52% ± 4.5%, for MVR, 37% ± 14% for AVR, and at 12 years of 52 ± 7.4% for MAVR. Embolic episodes occurred in 9 patients after AVR (0.7% ± 0.2% pt-yr), in 61 after MVR (1.7% ± 0.2% pt-yr), and in 6 after MAVR (1.7% ± 0.7% pt-yr); actuarial freedom from emboli at 15 years is 91% ± 3.5% after AVR, 79% ± 14% after MVR, and at 12 years is 87% ± 5% after MAVR. Reoperation because of primary tissue failure (PTF) was performed in 47 patients with AVR (3.9% ± 0.5% pt-yr), 91 with MVR (2.6% ± 0.3% pt-yr), and in 13 with MAVR (4.1% ± 1.1% pt-yr); actuarial freedom from PTF at 15 years is 41% ± 5.5% after MVR, 37% ± 10% after AVR, and at 12 years is 49% ± 13% after MAVR. After AVR and MVR, freedom from PTF is significantly better for patients over 50 years of age. HPB-related complications occurred in 80 patients with AVR (6.3% ± 0.7% pt-yr), 195 with MVR (5.5 ± 0.4 pt-yr), and in 41 with MAVR (12.0% ± 1.8% pt-yr); actuarial freedom all HPB-related complications at 15 years is 25% ± 4% after MTR, 23% ± 7.5% after ATR, and at 12 years is 20 ± 8.5% after MAVR. This extended follow-up of HPB recipients shows that the performance of this device declines progressively after the eighth postoperative year, becoming unsatisfactory beyond this limit mainly because of the increasing impact of PTF on HPB durability. These results justify our current trend to restrict HPB to a selected patient population.
A study of the conal anatomy of 36 specimens of complete transposition of the great arteries (TGA) is reported. The aim of the investigation was to verify if the two main anatomic features of the malformation, i.e. abnormal aorto-pulmonary and arterioventricular relationships, were strictly related to the maldevelopment of the conal structures. The findings indicate that in the cases with mitro-pulmonary fibrous continuity, there is a wide variation in subaortic conal development. and no correlation exists between the anterior position of the aorta and the length of the subaortic parietal conus. Therefore, differential conal absorption does not seem to play a role in the presumptive lack of rotation in TGA. TGA presenting with bilateral conus and biventricular pulmonary trunk origin are relatively frequent in this survey, but no correlation was observed between overriding of the pulmonary artery and development of the subpulmonary parietal conus. Consequently, in TGA the morphogenetic process of transfer of the pulmonary artery above the left ventricle does not seem to depend only on the absorption of the subpulomonary parietal conus. Ventricular septal defects (VSDs) in TGA are related frequently to conal pathology, either to some failure in conal septal growth or to malalignment of the normally developed conal and sinusal components of the ventricular septum.
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