Pulmonary hypertension in adults with congenital heart disease. Clinical phenotypes and outcomes in the advanced pulmonary vasodilator era.

BACKGROUND: Mortality of pulmonary hypertension associated with congenital heart disease (PAH-CHD) in adults remains high. OBJECTIVES: To identify predictors of death and to assess the impact of treatment on outcome. METHODS: Retrospective, multicenter cohort study of 103 adults with PAH-CHD followed-up for 8.6 ± 4.6 years. Patients were grouped according to underlying shunt type into pre-tricuspid, post-tricuspid and complex. Survival rates were analyzed and predictors of death were investigated with Cox regression models. RESULTS: In the post-tricuspid and complex groups (38 and 37 patients, respectively), the most common clinical PAH-CHD subgroup was Eisenmenger syndrome (76.3% and 59.5%, respectively) whereas, in the pre-tricuspid group (28 patients), 46.5% of patients had small or corrected defects. Overall, 88 patients received vasodilators; 39% required combination-therapy. Overall survival at 10 years was 65%. Mortality was highest in the pre-tricuspid group, FC-III-IV and amongst patients receiving monotherapy (p < 0.050). On multivariate analysis, predictors of poor outcome were pericardial effusion (HR: 4,520 [1,470-13,890]; p = 0,008), oxygen saturation(HR: 0.940 [0,900 - 0,990]; p = 0,018) and genetic syndromes(HR: 3,280 [1,098-9,780]; p = 0,033). CONCLUSIONS: Patients in advanced stages at initiation of treatment were at high risk of death and strong consideration should be given for more aggressive therapy.

Drug-eluting stents following rotational atherectomy for heavily calcified coronary lesions: long-term clinical outcomes.

BACKGROUND: Rotational atherectomy followed by drug-eluting stent (DES) implantation for complex, severely calcified lesions is a rational combination that has not been sufficiently evaluated. METHODS: We investigated 102 consecutive patients with angiographic evidence of heavily calcified lesions that underwent DES implantation following rotational atherectomy at our institution between June 2005 and October 2009, and we examined the long-term clinical outcomes. The major adverse cardiac events monitored were death, myocardial infarction and target lesion revascularization. RESULTS: Patients were 68.8 ± 7.4 years old, 52.9% were diabetic, and 12.7% had chronic kidney disease. Forty-seven patients (46.1%) had three-vessel disease, and 13 (12.7%) had left main coronary artery stenosis. The radial approach was used in 37.3% of cases. The procedure was successful in 97%. In-hospital death occurred in 1 patient (0.9%), and 3 patients (2.9%) developed stent thrombosis. At the mean follow-up period of 15 months (range 1- 54), the total cardiac death rate was 4.9%, target lesion revascularization was 8.8% and the incidence of myocardial infarction was 3.9%. The combined endpoint occurred in 12.7% of cases. CONCLUSION: DES following rotational atherectomy for heavily calcified coronary lesions is a safe and effective procedure that provides good long-term clinical outcomes.

Comparative findings of lymphocytic thyroiditis and thyroid lymphoma.

OBJECTIVE: To compare the cytologic features of histologically proven lymphocytic (Hashimoto's) thyroiditis (Hashimoto's thyroitidis) and primary thyroid lymphomas (TL). STUDY DESIGN: Clinical histories, smears (stained with Diff-Quik, Papanicolaou stain or hematoxylin and eosin [HE]) and surgical specimens (HE slides) were reviewed in 25 cases of lymphocytic thyroiditis and 12 of thyroid lymphomas. RESULTS: Surgical specimens of thyroiditis were obtained for other medical reasons: goiter and compressive symptomatology in 21 cases and neoplasms in 4 (2 papillary carcinomas, 1 follicular carcinoma and 1 oncocytic adenoma). Seven cases were primary lymphomas, and 5 were secondary. Histologically there were 6 large B-cell lymphomas, 2 mantle cell lymphomas, 1 Burkitt lymphoma, 2 mucosal-associated lymphoid tissue lymphomas in blastic transformation and 1 of unknown type. Sensitivity for the diagnosis was 67.5% for HT and 92.3% for lymphoma. CONCLUSION: A heterogeneous population of small and large lymphocytes was the most frequent pattern in both diseases. The presence of a monotonous population of large lymphocytes or, more rarely, of small cells indicates a probable TL. Plasma cells favor HT. Other techniques are mandatory for the differentiation of cases with inconclusive diagnoses.