Incidence of cancer and related deaths in hemoglobinopathies: A follow-up of 4631 patients between 1970 and 2021.

BACKGROUND: The correlation between thalassemia and malignancies other than hepatocellular carcinoma (HCC) and the possible relationship between other hemoglobinopathies and tumor risk have been poorly evaluated. METHODS: Eight Italian specialized centers evaluated the incidence of malignant neoplasms in hemoglobinopathies as well as their sites and features. The study cohort included 4631 patients followed between 1970 and 2021 (transfusion-dependent ß-thalassemia, 55.6%; non-transfusion-dependent thalassemia, 17.7%; sickle cell disease, 17.6%; hemoglobin H disease, 8.3%). RESULTS: A total of 197 diagnoses of cancer were reported (incidence rate, 442 cases per 100,000 person-years). The liver was the most frequent site of tumors in both sexes, with a higher incidence (190 cases per 100,000 person-years) in comparison with the general population found in all types of hemoglobinopathies (except hemoglobin H disease). In recent years, tumors have become the second cause of death in patients with transfusion-dependent thalassemia. A lower risk of breast and prostate cancer was observed in the whole group of patients with hemoglobinopathies. The first cancer diagnoses dated back to the 1980s, and the incidence rate sharply increased after the 2000s. However, although the incidence rate of cancers of all sites but the liver continued to show an increasing trend, the incidence of HCC showed stability. CONCLUSIONS: These findings provide novel insights into the relationship between cancer and hemoglobinopathies and suggest that the overall risk is not increased in these patients. HCC has been confirmed as the most frequent tumor, but advances in chelation and the drugs that have led to the eradication of hepatitis C may explain the recent steadiness in the number of diagnoses that is reported here.

[A late diagnosis of left atrial myxoma: how to gain time?] / Una diagnosi tardiva di mixoma atriale sinistro. Come guadagnare tempo?

A 56-year-old man presented to the Outpatient Cardiology Unit for dyspnea that had been lasting 6 months and an occasional episode of cold perspiration on climbing a flight of stairs. In the suspicion of coronary artery disease, he was prescribed a complete blood panel, an echocardiogram and a treadmill stress test. The echocardiogram, performed as late as 78 days after the first evaluation and only by chance scheduled 2 days before the stress test, enabled a diagnosis of left atrial myxoma for which the patient successfully underwent cardiac surgery. The authors discuss the aspecific and potentially misleading nature of myxoma symptoms and highlight the latency between cardiological evaluation and diagnostic echocardiography.

Coronary artery bypass graft and mitral valvuloplasty in a patient with isolated ventricular non-compaction.

A case of isolated ventricular non-compaction associated to three-vessel disease and a mitral regurgitation is described. The patient underwent triple coronary artery bypass graft and restrictive mitral annuloplasty. The postoperative course was unsuccessful despite the very depressed left ventricular (LV) function. At two years follow-up, no major adverse cardiac event has occurred and the LV function was slightly improved.

Predictors of adverse events after surgical ventricular restoration for advanced ischaemic cardiomyopathy.

OBJECTIVE: Post-infarction ventricular remodelling has been graded (I-III) according to the loss of systolic left ventricular silhouette curvature changes. Although surgical ventricular restoration (SVR) has been extended to type III ischaemic cardiomyopathy, the results are less satisfactory. We sought to identify geometric and functional predictors of late outcome after SVR. METHODS: Among 144 patients who underwent SVR since 1998, a subgroup of 31 patients (age: 65.2+/-7.6 years) was analysed. Inclusion criteria were: type III cardiomyopathy, no associated procedure except coronary artery bypass grafting, prior anterior infarction, absent-to-2+ mitral regurgitation, elective operation, follow-up > or =18 months (mean: 44+/-26; longest: 96 months). Probability of events was estimated with the Kaplan-Meier method. A Cox multivariable regression model was constructed selecting eight potential predictors of four adverse events: death, cardiac death, recurrent heart failure (New York Heart Association class III or IV) and left ventricular re-remodelling, defined as a 25% increase of end-systolic volume index after SVR, or an end-systolic volume index > or =50 ml.m(-2). RESULTS: Early and late mortality were zero and 6% (2/31 patients, one cardiac-related death). NYHA class and all echocardiographic functional variables significantly improved early after SVR. Freedom (+/-standard error (SE)) from heart failure was 97%+/-3%, 93%+/-5%, 77%+/-11% and 64%+/-15%, whereas freedom from left ventricular re-remodelling was 97%+/-3%, 80%+/-8%, 60%+/-12% and 39%+/-15%, respectively, 1, 3, 5 and 7 years after SVR. Multivariable analysis identified baseline mitral regurgitation degree and sphericity index as independent predictors of recurrent heart failure (p=0.025; hazard ratio (HR)=7.80 (95% confidence intervals (CIs): 1.29-47.19)) and left ventricular re-remodelling (p=0.047; HR=2.84 (95% CIs: 1.01-7.95)). Both predictors also correlated with a higher recurrence of end-systolic volume index > or =50 ml.m(-2) at late follow-up. CONCLUSIONS: Despite advanced cardiomyopathy, SVR determines left ventricular volume reduction and improved systolic function. Baseline absent-to-moderate mitral regurgitation and a more spherical left ventricular geometry predict a less favourable clinical and functional outcome, suggesting a possible rationale for wider indications for combined correction of 2+ mitral regurgitation and undersizing of the mitral annulus, particularly in patients with sphericity index > or =0.75.

Baseline left ventricular function and surgical annular stiffening to predict outcome and reverse left ventricular remodeling after undersized annuloplasty for intermediate-degree ischemic mitral regurgitation.

OBJECTIVE: We sought to identify determinants of clinical and functional outcome after myocardial revascularization and associated undersized annuloplasty in patients with intermediate-degree ischemic mitral regurgitation. METHODS: Fifty-seven patients with 2+ or 3+ ischemic mitral regurgitation underwent coronary bypass surgery and implantation of undersized semirigid or flexible complete ring or autologous pericardial band and were followed up to 8.6 years. RESULTS: Operative mortality was 5%. Baseline left ventricular end-systolic volume index, the strongest multivariable predictor of early postoperative outcome, was correlated with end-systolic volume index (P < .001, R(2) = 0.67) and ejection fraction (P < .001, R(2) = 0.40) after repair. More compromised ejection fraction and end-systolic volume index predicted comparatively greater early functional improvement but higher residual postoperative end-systolic volume index (P < .01). Cox multivariable analysis identified wall motion as the best baseline predictor of late death and heart failure and regional inferoposterior wall motion as the strongest predictor of recurrent mitral regurgitation (P < or = .01). More rigid annuloplasty carried a higher probability of functional recovery in terms of ejection fraction, wall motion, and the occurrence and earlier timing of left ventricular reverse remodeling, expressed by different degrees of end-systolic volume index reduction (P < .001, hazard ratio >6). CONCLUSIONS: Combination of undersized mitral annuloplasty and coronary revascularization presents low operative mortality and determines left ventricular unloading in patients with intermediate-degree ischemic mitral regurgitation. Global and regional wall motion are powerful predictors of late outcome. Stiffer mitral annular repair promotes functional recovery and predicts higher probability and earlier timing of reverse remodeling.

Off-pump coronary artery bypass grafting without mechanical stabilization in minipericardiotomy.

Stabilization and exposure of coronary vessels in off-pump bypass grafting is achieved by means of different and expensive mechanical stabilizers. We describe a new technique of stabilization and exposure of target vessels by using only a 'double suspension-stabilization stitch' without the support of mechanical stabilizers. In accomplishing left anterior descending and right coronary artery off-pump bypass grafting, this technique has resulted in a good hemodynamic stability, in no epicardial damage and surgical trauma, and finally it represents a costless procedure.

Off-pump coronary artery bypass grafting without mechanical stabilization in minipericardiotomy: a preliminary experience.

We describe a new off-pump coronary bypass technique, the "double suspension-stabilization stitch", performed without the support of mechanical stabilizers. This technique is very cheap and yields good hemodynamic stability, with no epicardial damage.