Matilde Nardi

Effects of short- and long-term carvedilol administration on rest and exercise hemodynamic variables, exercise capacity and clinical conditions in patients with idiopathic dilated cardiomyopathy

OBJECTIVES The study evaluated the effects of short- and long-term administration of carvedilol in patients with idiopathic dilated cardiomyopathy. BACKGROUND Carvedilol is a beta-adrenergic blocking agent with vasodilator activity that might be well tolerated in patients with heart failure. METHODS Forty patients with idiopathic dilated cardiomyopathy treated with digoxin, furosemide and angiotensin-converting enzyme inhibitors were randomized in a double-blind manner to receive either placebo or carvedilol. Right heart hemodynamic variables were evaluated up to 8 h after short-term drug administration and, on the next day, during cardiopulmonary exercise testing before and 3 h after drug ingestion. Placebo or carvedilol was added to standard therapy, starting with a dose of 6.25 mg twice a day with weekly increments up to the maximum of 25 mg twice a day. Patients were reevaluated after 4 months by cardiopulmonary exercise testing and measurement of right heart hemodynamic variables 12 h after last drug ingestion and 3 h after drug readministration. Left ventricular ejection fraction and volume, measured by equilibrium radionuclide ventriculography, quality of life and submaximal exercise duration were assessed before and after long-term therapy. RESULTS Compared with placebo, carvedilol produced a short-term reduction in heart rate and pulmonary artery and pulmonary wedge pressures and, after long-term administration, increased both rest and peak exercise cardiac, stroke volume and stroke work indexes, with a further reduction in right atrial, pulmonary artery and pulmonary wedge pressures. Long-term carvedilol administration also improved rest left ventricular ejection fraction (from 20 +/- 7% to 30 +/- 12%, p < 0.001), submaximal exercise capacity, quality of life and New York Heart Association functional class. No baseline variable was predictive of the response to therapy. CONCLUSIONS Short-term carvedilol administration reduces heart rate and mean pulmonary artery and pulmonary wedge pressures, whereas it improves both long-term rest and exercise left ventricular systolic function, reduces heart failure symptoms and improves submaximal exercise tolerance in patients with idiopathic cardiomyopathy.

Tubulointerstitial nephritis is a dominant feature of hereditary apolipoprotein A-I amyloidosis.

Apolipoprotein A-I is the main protein of high-density lipoprotein particles, and is encoded by the APOA1 gene. Several APOA1 mutations have been found, either affecting the lecithin:cholesterol acyltransferase activity, determining familial HDL deficiency, or resulting in amyloid formation with prevalent deposits in the kidney and liver. Evaluation of familial tubulointerstitial nephritis in patients with the Leu75Pro APOA-I amyloidosis mutation resulted in the identification of 253 carriers belonging to 50 families from Brescia, Italy. A total of 219 mutation carriers underwent clinical, laboratory, and instrumental tests. Of these, 62% had renal, hepatic, and testicular disease; 38% were asymptomatic. The disease showed an age-dependent penetrance. Tubulointerstitial nephritis was diagnosed in 49% of the carriers, 13% of whom progressed to kidney failure requiring dialysis. Hepatic involvement with elevation of cholestasis indices was diagnosed in 30% of the carriers, 38% of whom developed portal hypertension. Impaired spermatogenesis and hypogonadism was found in 68% of male carriers. The cholesterol levels were lower than normal in 80% of the mutation carriers. Thus, tubulointerstitial nephritis was highly prevalent in this large series of patients with Leu75Pro apoA-I amyloidosis. Persistent elevation of alkaline phosphatase, reduced HDL cholesterol plasma levels, and hypogonadism in men are key diagnostic features of this form of amyloidosis.

Persistence of left superior vena cava, absence of coronary sinus and cerebral ictus

Persistence of left superior vena cava (LSVC) is the most frequent venous thoracic congenital anomaly. Right superior vena cava (RSVC) develops, during embryogenesis, from right anterior cardinal vein, while left anterior cardinal vein atrophies [1]. If left anterior cardinal vein persists during embryogenesis, persistence of LSVC occurs. This anomaly occurs in 0.3–0.5% of the normal population and in 1.3– 10% of patients with cardiac malformations including anomalus drainage of left pulmonary veins, hypoplasia of left-sided structures (e.g. mitral stenosis or coarction), and other complex congenital heart disease [2,3]. RSVC is absent in 1% of patients with persistence of LSVC [9]. Communication between LSVC and RSVC may be present through innominate vein. Absence of coronary sinus is a rare eventuality in persistence of LSVC [7,8], usually associated with interatrial defect [7,9]. Persistence of LSVC usually drains into right atrium throw coronary sinus (92%) or directly into left atrium (8%, unroofed coronary sinus). The latter may lead to left-to-right shunt and cyanosis or to paradox embolism [4,5]. Isolated persistent LSVC is usually not recognized until left cephalic or subclavian approach is used for diagnostic and therapeutic transcatheter procedures. Coronary sinus drainage assumes importance in case of

Coronary-to-bronchial anastomosis: an unusual cause of hemoptysis.

Coronary-to-bronchial anastomosis (CBA) is a rare anomaly. This vascular abnormality may be subclinical or be responsible for several pathophysiological events and symptoms involving the respiratory and/or the coronary system. We report the case of a patient with hemoptysis caused by this anomalous coronary-to-bronchial communication, who was concomitantly affected by aortic stenosis and coronary artery disease requiring surgical treatment. A coronary angiogram clearly demonstrated the abnormal vascular connection between the proximal right coronary artery and the bronchial arteries of the left inferior right lobe. The coronary branch was intra-operatively identified and ligated, in association with aortic valve replacement and coronary artery bypass, with an uneventful postoperative course and resolution of the respiratory symptoms. This case presents an unusual cause of hemoptysis due to CBA in patients with other cardiac comorbidities, which required surgical treatment, allowing direct ligation of the CBA. A review of the clinical and therapeutic characteristics of such a peculiar vascular abnormality is also provided.

Role of different vascular approaches on transcatheter aortic valve implantation outcome: a single-center study.

Objective To compare different vascular approaches on clinical outcome of patients undergoing transcatheter aortic valve implantation (TAVI) with self-expandable bioprosthesis. Methods We included all the patients undergoing CoreValve implantation at our institute between September 2007 and March 2014. They were divided into four groups based on the vascular approach: percutaneous transfemoral (pTF), cut-down transfemoral (cTF), transaxillary (TAx) and transaortic (TAo). Clinical outcomes were evaluated according to Valve Academic Research Consortium-2 recommendations. Results Out of 322 consecutive patients, 170 (53%) underwent pTF, 76 (23%) cTF, 32 (10%) TAx and 44 (14%) TAo approach. Although the TAx and TAo patients had a higher risk profile, they had a similar outcome compared with the pTF and cTF groups; in particular, there were no differences regarding cardiovascular and all-cause mortality at 30 days, 1 and 2 years, as well as stroke, myocardial infarction, bleeding, major vascular complications, permanent pacemaker implantation and acute kidney injury rates. The observed device success rate was higher in the TAo than in the other approaches (88.6 versus 65.9, 68.7 and 76.3% in the pTF, cTF and TAx groups, respectively; P = 0.019). No differences occurred regarding 30-day early safety and 1-year clinical efficacy across the four groups. Fluoroscopy time, amount of contrast medium used and minor vascular complications were significantly higher in pTF patients, as well as in-hospital stay in the TAo group. Atrial fibrillation and prosthetic valve regurgitation, but not the vascular approach, were independent predictors of all-cause mortality. Conclusion A more invasive vascular approach, for CoreValve implantation, even in higher risk patients, does not affect early-term, mid-term and long-term outcomes.

Edge-to-edge mitral valve repair for isolated prolapse of the anterior leaflet caused by degenerative disease.

Objective The use of new techniques to correct mitral regurgitation due to prolapse of the anterior leaflet has been shown to expand the original surgical armamentarium and to improve postoperative outcome. We retrospectively analysed our experience with isolated prolapse of the anterior mitral leaflet repaired using the edge-to-edge technique. Methods From October 1986 to June 2004, 790 patients underwent mitral valve repair for mitral regurgitation at our institution. Isolated pathology of the anterior mitral leaflet, due to degenerative disease, was the cause of mitral regurgitation in 84 patients and, from 1991, 68 underwent edge-to-edge repair. Results There was no intraoperative death and one in-hospital death. Three patients died in the late follow-up period for a cumulative 13-year survival rate of 90 ± 1.4%. No patient underwent early reoperation. Four patients underwent reoperation during the follow-up for a cumulative 13-year freedom from reoperation of 92.3 ± 3.2%. At echocardiographic evaluation, mitral valve repair failure was associated with severe mitral regurgitation in four patients. Of the remaining 60 patients, 40 had no residual regurgitation, 18 had trivial residual regurgitation, and two had mild regurgitation. At follow-up, 49 patients are still in New York Heart Association (NYHA) class I, 14 in NYHA class II and only one in NYHA class III. Conclusions Our study demonstrates that the ‘edge-to-edge’ technique is a reliable procedure to correct prolapsing leaflets. The addition of this technique to the surgical armamentarium has neutralized prolapse of the anterior leaflet as an incremental risk factor for reoperation.

Triple-orifice valve repair in severe Barlow disease with multiple-jet mitral regurgitation: Report of mid-term experience

OBJECTIVES Barlow disease represents a surgical challenge for mitral valve repair (MR) in the presence of mitral insufficiency (MI) with multiple regurgitant jets. We hereby present our mid-term experience using a modified edge-to-edge technique to address this peculiar MI. METHODS From March 2003 till December 2010, 25 consecutive patients (mean age 54 ± 7 years, 14 males) affected by severe Barlow disease with multiple regurgitant jets were submitted to MR. Preoperative transesophageal echo (TEE) in all the cases showed at least 2 regurgitant jets, involving one or both leaflets in more than one segment. In all the patients, a triple orifice valve (TOV) repair with annuloplasty was performed. Intra-operative TEE and postoperative transthoracic echocardiography (TTE) were carried out to evaluate results of the TOV repair. RESULTS There was no in-hospital death and one late death (non-cardiac related). At intra-operative TEE, the three orifices showed a mean total valve area of 2.9 ± 0.1cm(2) (range 2.5-3.3 cm(2)) with no residual regurgitation (2 cases of trivial MI) and no sign of valve stenosis (mean transvalvular gradient 4.6 ± 1.5 mmHg). At follow up (mean 38 ± 22 months), TTE showed favourable MR and no recurrence of significant MI (6 cases of trivial and 1 of mild MI). Stress TTE was performed in 5 cases showing persistent effective valve function (2 cases of trivial MI at peak exercise). All the patients showed significant NYHA functional class improvement. CONCLUSIONS This report indicates that the TOV technique is effective in correcting complex Barlow mitral valves with multiple jets. Further studies are required to confirm long-term applicability and durability in more numerous clinical cases.

Tricuspid valve myxoma in a patient with congestive heart failure

Myxomas are the most frequent benign primary cardiac tumours (50% of benign heart tumours). This kind of tumour is most likely to be localized in the left atrium, followed by the right atrium, right ventricle and left ventricle. Quite exceptional is the presence of a myxoma originating from the tricuspid valve or from the Eustachian valve. We describe the case of a woman with moderate dyspnoea of unknown origin and the presence of tricuspid myxoma who underwent tricuspid valve curettage.

Prognostic Significance of Change in the Left Ventricular Ejection Fraction After Transcatheter Aortic Valve Implantation in Patients With Severe Aortic Stenosis and Left Ventricular Dysfunction

Patients with severe aortic stenosis and reduced left ventricular ejection fraction (LVEF) have a poor prognosis compared with patients with preserved LVEF. To evaluate the impact of early LVEF recovery in patients with baseline dysfunction on clinical outcomes after transcatheter aortic valve implantation (TAVI), we included all consecutive patients who underwent TAVI from the Italian ClinicalService registry with an LVEF of ≤45% at baseline who had 1-month LVEF data. Patients who experienced a previous coronary artery bypass graft, a previous valve replacement, or a previous myocardial infarction were excluded from the analysis. Therefore, 131 patients with an improvement in LVEF of <10% (no-R group) were compared with 121 patients with an improvement in LVEF of ≥10% (R group). The primary end point was the rate of death of any cause. Multivariable analysis was performed to determine independent predictors of lack in LVEF recovery. Early LVEF recovery occurred in 48% of the patients, generally before discharge. One-year all-cause mortality and major adverse cardiac and cerebrovascular events were significantly higher in the no-early recovery group (log rank test p = 0.005 and p = 0.003, respectively). Baseline severe left ventricular dysfunction and previous percutaneous coronary intervention were identified as independent predictors to warn the lack of improvement in LVEF. In conclusion, nearly 50% of patients with preoperative left ventricular dysfunction demonstrated a significant early improvement in LVEF after TAVI. Lack of early LVEF recovery is associated with a worse clinical outcome and is most likely among patients with a severely abnormal baseline LVEF and a previous percutaneous coronary intervention.

Giant asymptomatic left atrial myxoma

The classic triad of myxoma clinical presentation is characterized by intracardiac obstruction, embolisms, and constitutional symptoms with fever, weight loss, or symptoms resembling connective tissue disease. Giant myxoma without symptoms are very rare. We present a case of a 30-year-old female with a giant asymptomatic myxoma in the left atrium, discovered by echocardiography. The patient was asymptomatic.