IgG4-related disease and ANCA positive vasculitis in childhood: a case-based review

Clinical Rheumatology - Autoimmune pancreatitis (AIP) type 1 is an IgG4-related disease (IgG4-RD), characterized by inflammatory pseudotumors and histologically by dense lymphoplasmacytic...     

Coronary lesions due to aortic valve disease. II -- Results and indications of aortocoronary bypass surgery]

454 cases of aortic valve replacement were studied: 217 had no significant coronary artery disease, 197 had associated aorto-coronary bypass surgery and 40 coronary artery disease without revascularization surgery:-- The early mortality in the three groups was 5.5%, 21% and 12.5% respectively, the only statistically significant difference being between the first two groups. -- The coronary artery disease was signigicantly more severe in the group which underwent associated aorto-coronary bypass surgery. The early mortality was significantly raised in the group without bypass surgery in cases with severe coronary artery disease (28%) and in the group with bypass surgery with unsuitable coronary artery lesions (35.5%). On the other hand, the long term survival and functional capacity of patients who underwent associated bypass surgery approached that of the non-coronary patients. Combined aortic valve replacement and coronary bypass surgery should therefore be continued in selected cases.     

Aorto-right ventricular shunt and associated aortic valve injury resulting from a penetrating wound of the heart

A case of a traumatic aorto-right ventricular fistula associated with an aortic valve injury and subsequent successful treatment is reported. A possible delay in diagnosis necessitates follow-up of patients after penetrating thoracic wounds. Early surgical treatment is mandatory because of the risks of both heart failure and endocarditis and because of the low operative morbidity. An aortic approach appears to be preferable because it permits repair of both the fistula and the valvular injury. Reconstruction of valvular injuries is preferable since long-term competence without prosthetic replacement can be achieved.     

Abnormal implantation of a coronary artery in a 53-year-old patient. Surgical correction

In a 53-years old patient complaining of dyspnoea and angina of effort, the coronary arteriography demonstrated ab abnormal implantation of the pulmonary artery into the left coronary-artery. Correlated with the 39 cases of the adolescent and the adult published in the literature, the case reported has some peculiarities: poorness of the auscultatory signs consisting in a mild apical systolic murmur; electrocardiographic pattern of left complete bundle-branch block; presence of massive calcifications visible by X-ray into the lateral, wall of the left ventricle. Coronary arteriography and catherization made it possible to demonstrate a left-to-right shunt by backward-flow revascularization of the left coronary artery starting from the right coronary artery. A simple suture of the implantation foramen of the abnomal coronary artery resulted in increased pressure into this artery and was followed by disappearance of angina during an observation period of 5 months.     

Use of 2 internal mammary arteries in coronary bypass. Combination with a bypass using the gastroepiploic artery in 2 cases

Coronary revascularisation by bilateral internal mammary artery grafting was performed in 39 patients. The left internal mammary was anastomosed to the left anterior descending (N = 22), the left lateral (N = 12) or diagonal artery (N = 5). The right internal mammary was implanted on the right coronary (N = 17), the left anterior descending (N = 16), the left lateral (N = 3) or diagonal artery (N = 3). In two cases, the inferior myocardial segments were revascularised by grafting the gastroepiploic artery. Three patients died and one patient had signs of postoperative myocardial infarction. Respiratory complications, usually mild, occurred in 5 patients. Bilateral internal mammary artery grafting should be reserved for: 1) young patients, 2) those without usable leg veins (varicose veins, previous stripping or coronary bypass surgery). The gastroepiploic artery is a useful arterial graft for revascularizing regions of the heart inaccessible to the internal mammary arteries.     

Assessment of atrial septal defect size with 3D-transesophageal echocardiography: comparison with balloon method

BACKGROUND: Transcatheter closure of atrial septal defect (ASD) is an alternative approach to surgery in selected patients. Balloon stretched diameter (BSD) is considered as the standard way of measuring ASD size. Three-dimensional transesophageal echocardiography (3D-TEE) provides views of the ASD allowing its measurement and identifying its spatial relation with neighboring structures. Our aim was to compare the BSD and 3D-TEE methods to measure the ASD size before transcatheter closure. METHODS AND RESULTS: Seventy-six consecutive patients were enrolled for ASD device closure. Three-dimensional transesophageal echocardiography and balloon sizing were adequately performed in 70 patients before the defect closure. The mean maximal diameter measured by 3D-TEE was 20 +/- 15 mm (range 10-28) while the mean BSD was 22 +/- 4.8 mm (range 9-31). When comparing the 3D-TEE and transcatheter measurements, there was a good correlation between the two methods (y = 3.15 + 0.77x; r = 0.8). The defect as viewed by 3D-TEE was unique in 54 patients and multiple in 16 patients. In patients with a single defect, the correlation between the two methods was high (y = 1.74 + 0.84x; r = 0.85) while patients with multiple ASDs, the correlation was poor (y = 12.4 + 0.4x; r = 0.45). Transcatheter closure was performed successfully in 86%. The mean size of the Amplatzer device was 23 +/- 4.8 mm (range 4-32). The reference to choose the size of the device was the BSD in single defects and the 3D-TEE maximal diameter in multiple defects. CONCLUSION: Three-dimensional transesophageal echocardiography and transcatheter methods are two complementary techniques for the success of transcatheter ASDs closure.     

Cardiovascular abnormalities in Turner's syndrome: What prevention?

Cardiovascular complications in Turner's syndrome are the most common cause of excess early mortality, with a life expectancy that may be reduced by more than 10 years. Congenital cardiac abnormalities are described in approximately one third of patients. These abnormalities are mostly left heart obstructions, the most common of which are bicuspid aortic valve (16%) and coarctation of the aorta (11%). Dilatations of the ascending aorta are often described and may occur in isolation from any heart disease, suggesting a vasculopathy specific to the syndrome, probably predisposed to by extracardiac risk factors such as oestrogen deficiency, diabetes, dysplidaemia and overweight. The most feared complication is aortic dissection with around a 100 cases, described at average age of approximately 35-years-old. This is believed to complicate 2% of induced pregnancies. Hypertension (HBP) usually essential, affects up to 50% of patients with Turner's syndrome. This is an important risk factor for cardiovascular complications and justifies aggressive treatment. On the other hand, retrospective studies have not demonstrated adverse cardiological effects due to growth hormone treatments. Patients with Turner's syndrome merit regular cardiology follow-up from childhood onwards, particularly if they have treated heart disease. The merits of preventative treatments for aortic dilatation have not been demonstrated in Turner's syndrome and justify prospective trials.