Pitfalls in arrhythmogenic left ventricular cardiomyopathy (ALVC). A review of the literature with considerations on a single case of sudden death in a juvenile athlete.

Sudden cardiac death (SCD) in young athletes represents a challenging issue in forensic practice. The pathologist is frequently asked to establish the cause of death basing upon anatomical findings and to evaluate the role of the physician in preparticipation evaluation (PPE) and eligibility decision. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of SCD during sport activity. However, in the last few years, forms with predominant or even isolated involvement of the left ventricle (LV) have progressively been correlated with a high risk of SCD. We present a case of SCD in an apparently healthy 19-year-old semi-professional football player. Annual PPEs performed in accordance with international and Italian recommendations, were unremarkable. At autopsy, a 1-cm area of subepicardial fibro-fatty replacement was observed at the postero-lateral wall of the LV. The finding was diagnostic of arrhythmogenic left ventricular cardiomyopathy (ALVC). A review of this rare pathology has been performed under a forensic perspective, focusing on the evaluation of the medico-legal responsibility of the physician in the PPE and on the morphological aspects of the disease. Current diagnostic criteria and recommendations result to be focused on the right ventricular pattern, with a risk of misdiagnosis for isolated LV forms. Furthermore, few detailed autopsies cases concerning ALVC have been published. There is a need, therefore, to study this rare disease with a careful and revised approach.

Right Ventricular Strain and Dyssynchrony Assessment in Arrhythmogenic Right Ventricular Cardiomyopathy: Cardiac Magnetic Resonance Feature-Tracking Study.

BACKGROUND: Analysis of right ventricular (RV) regional dysfunction by cardiac magnetic resonance (CMR) imaging in arrhythmogenic RV cardiomyopathy (ARVC) may be inadequate because of the complex contraction pattern of the RV. Aim of this study was to determine the use of RV strain and dyssynchrony assessment in ARVC using feature-tracking CMR analysis. METHODS AND RESULTS: Thirty-two consecutive patients with ARVC referred to CMR imaging were included. Thirty-two patients with idiopathic RV outflow tract arrhythmias and 32 control subjects, matched for age and sex to the ARVC group, were included for comparison purpose. CMR imaging was performed to assess biventricular function; feature-tracking analysis was applied to the cine CMR images to assess regional and global longitudinal, circumferential, and radial RV strains and RV dyssynchrony (defined as the SD of the time-to-peak strain of the RV segments). RV global longitudinal strain (-17±5% versus -26±6% versus -29±6%; P<0.001), global circumferential strain (-9±4% versus -12±4% versus -13±5%; P=0.001), and global radial strain (18 [12-26]% versus 22 [15-32]% versus 27 [20-39]%; P=0.015) were significantly lower and SD of the time-to-peak RV strain in all 3 directions were significantly higher among patients with ARVC compared with patients with RV outflow tract arrhythmias and controls. RV global longitudinal strain >-23.2%, SD of the time-to-peak RV longitudinal strain >113.1 ms, and SD of the time-to-peak RV circumferential strain >177.1 ms allowed correct identification of 88%, 75%, and 63% of ARVC patients with no or only minor CMR criteria for ARVC diagnosis. CONCLUSIONS: Strain analysis by feature-tracking CMR helps to objectively quantify global and regional RV dysfunction and RV dyssynchrony in patients with ARVC and provides incremental value over conventional cine CMR imaging.

Disease-specific differences of left ventricular rotational mechanics between cardiac amyloidosis and hypertrophic cardiomyopathy.

Left ventricular (LV) twist (LVT) and untwisting (LVUT) rate are global and thorough parameters of LV function. The aim of the present study was to investigate the differences in LV rotational mechanics between patients with cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM). Twenty consecutive patients with CA, 20 consecutive patients with HCM, and 20 consecutive subjects without evidence of structural heart disease were included. Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging was performed to evaluate biventricular function, LV mass index, and presence/extent of LGE. Feature-tracking analysis was applied to LV basal and apical short-axis images to determine peak LVT, time to peak LVT, peak LVUT rate, and time to peak LVUT rate. Peak LVT and peak LVUT rate were significantly impaired in patients with CA compared with controls (P < 0.05 for both). In patients with HCM, peak LVT was increased (P < 0.05) compared with controls, whereas peak LVUT rate was preserved (P > 0.05). Time to peak LVUT rate was significantly prolonged in patients with CA and in patients with HCM compared with controls (ANOVA P < 0.001). At multivariate analysis, age (P = 0.007), LV ejection fraction (P = 0.035) and extent of LGE (P < 0.001) were independently related to peak LVT, and LV mass index (P = 0.015) and extent of LGE (P = 0.004) were independently related to peak LVUT rate, whereas extent of LGE (P < 0.001) was the only variable independently related to time to peak LVUT rate. In conclusion, CA and HCM have specific behavior of LV rotational mechanics. The extent of LGE significantly influences the LV rotational mechanics.

Patient-prosthesis mismatch after small-size stentless aortic valve replacement.

OBJECTIVE: The aim of this study was to determine the occurrence of patient-prosthesis mismatch (P-PM) after aortic valve replacement (AVR) with a small-size Cryolife O'Brien (CLOB) bioprosthesis and to evaluate its clinical and hemodynamic implications. METHODS: Sixty-two patients (mean age 70.9 +/- 5.2 years, 77.8% females), receiving a labeled 21-23 mm CLOB between 1993 and 2000, were retrospectively studied. Effective orifice area (EOA) was calculated by the continuity equation and then indexed to the patient's body surface area (BSA) to obtain the indexed EOA (EOAI). Based on previous observations a mismatch was defined as EOAI or= 0.8 cm/m2 showed an earlier concentric remodeling up to 1 year; no difference was demonstrated at later studies between groups. Survival and clinical status results were not affected by an EOAI

Sixteen-year results of composite aortic root replacement for non-dissecting chronic aortic aneurysms.

BACKGROUND: The aim of this study was to evaluate the early and long-term outcomes in patients undergoing a Bentall procedure or its button modification for chronic aortic aneurysms with aortic valve incompetence. METHODS: Between January 1986 and January 2002, 65 patients (84% males, mean age 58.9 +/- 11 years) underwent aortic root replacement with a Bentall or a button-Bentall operation. Annuloaortic ectasia was the most frequent cause of aortic disease in this series (n = 37, 56.9%), followed by atherosclerotic aneurysms (n = 22, 33.9%), and post-stenotic dilation (n = 5, 7.7%). One patient (1.5%) underwent redo aortic root replacement,3 (4.6%) had a Marfan syndrome, and 6 (9.2%) underwent a concomitant replacement of the aortic arch. The duration of follow-up ranged from 2 to 192 months (mean 89.6 +/- 21.8 months). RESULTS: The 30-day mortality was 0%. Early non-fatal complications comprised: bleeding requiring surgical re-exploration (n = 1, 1.5%), low output syndrome (n = 1, 1.5%), acute renal insufficiency (n = 1, 1.5%), transient ischemic attack (n = 2, 3.1%), stroke (n = 1, 1.5%), and pulmonary insufficiency (n = 1, 1.5%). There was a late death due to a pulmonary neoplasm. The 16-year actuarial survival was 97 +/- 2% (hazard 0.02 +/- 0.02). No patient required reoperation. Furthermore, the long-term clinical follow-up was characterized by the complete absence of endocarditis, anticoagulant-related hemorrhage, valve thrombosis, and prosthetic failure. Finally, the NYHA functional status was significantly improved (1.1 +/- 0.50, p < 0.001 vs preoperatively). CONCLUSIONS: The late results of the Bentall and button-Bentall procedures were excellent. Our findings confirm that these techniques still constitute the gold standard in the surgical treatment of combined valve and ascending aorta pathologies.

Long-term results of Bentall composite aortic root replacement for ascending aortic aneurysms and dissections.

STUDY OBJECTIVES: The aim of this study was to evaluate the early and long-term outcomes in patients undergoing aortic root replacement (ARR) with the Bentall procedure. DESIGN: Retrospective study. SETTING: Cardiothoracic surgery unit. PATIENTS AND METHODS: Between January 1986 and January 2002, 72 patients (mean age 58.3 +/- 12.4 years, 81.9% males) underwent ARR by means of a Bentall operation. Annuloaortic ectasia was the most frequent cause of aortic disease in this series of patients (31 patients; 43.1%), followed by type A dissection (19 patients; 26.3%), atherosclerotic aneurysm (18 patients; 25.1%), and poststenotic dilatation (4 patients; 5.5%). Nine patients (12.5%) had Marfan syndrome, and 10 patients (13.8%) underwent a concomitant replacement of the aortic arch. Follow-up ranged from 2 to 192 months (mean [+/- SD], 86.6 +/- 23.8 months). RESULTS: The mean 30-day mortality rate was 5.5 +/- 2%. The mean early mortality rate was 21 +/- 4% and 0% (p < 0.001), respectively, in patients with and without dissecting aortic aneurysms. There were two late deaths that were due to a pulmonary neoplasm and a cerebrovascular accident. The mean 16-year survival rate was 91.7 +/- 3.2%. The mean hazard of freedom from death was constant beyond 3 years (8.5 +/- 3.5%). No patient required reoperation. Furthermore, the long-term clinical follow-up was marked by a complete absence of endocarditis, anticoagulant-related hemorrhage, valve thrombosis, and prosthesis failure. Finally, patients showed a significant improvement in mean New York Heart Association functional status (1.3 +/- 0.1; p < 0.001 [postoperatively vs preoperatively]). CONCLUSIONS: In our experience, the late results of the Bentall operation were satisfactory. Our findings confirm that this technique still represents the procedure of choice for ARR with coronary reimplantation.

Early in vivo hemodynamic results after aortic valve replacement with the St Jude Medical Regent mechanical heart valve in patients with pure aortic stenosis.

BACKGROUND: The St Jude Medical Regent is a new-generation mechanical heart valve that represents a design evolution of the St Jude Hemodynamic Plus Series (HP). The purpose of this study was to evaluate early "in vivo" hemodynamic performance of the Regent valve in patients with aortic stenosis. METHODS: Between March 2000 and December 2001, 32 patients (mean age 59.9 +/- 5.9,56.3% male) with pure aortic stenosis received a Regent mechanical prosthesis in the aortic position. Hemodynamic performance was assessed by Doppler echocardiography at discharge, two months, six months, and one year by calculating peak transprosthetic velocity (Vmax), mean (MG) and peak (PG) transprosthetic gradients, effective orifice area index (EOAI), left ventricular mass index (LVMI), and degree of aortic regurgitation. RESULTS: A significant reduction in mean and peak transaortic gradients (p < 0.001) and a significant increase in EOAI (p < 0.001) over time followed valve replacement, and a bivariate analysis of variance (ANOVA) failed to demonstrate statistical differences by valve size over time (p = ns). A significant reduction in left ventricular hypertrophy occurred over time (p < 0.001) in all valve sizes (p = ns between groups): baseline LVMI was 221 +/- 57 g/cm2; it decreased by 30 g/cm2 (p < 0.001) at discharge. LVMI decreased from 191 +/- 54 g/cm2 to 161 +/- 41 g/cm2 (p < 0.001) from discharge to two months. Further reductions were not significant. At the six-month follow-up no patient in our cohort exhibited moderate or severe aortic regurgitation at Doppler echocardiography. CONCLUSIONS: Early results with the St Jude Medical Regent valve have been satisfactory. Further assessments are necessary to confirm these results.

A short-term experience with the Tirone David I valve sparing operation for the treatment of aneurysms of the ascending aorta and aortic root.

AIM: The aim of this report is to summarize our experience with the Tirone David I technique over a 4-year period. METHODS: Since 1997 15 patients were operated on using this surgical procedure. Of the patients, nine were male (60%) and six (40%) female. Ages ranged from 23 to 75 years (mean 62.1+/-17.2 years). The underlying disease was chronic aneurysm of the ascending aorta or root in 13 (86.6%) and chronic dissection in two (13.4%). Four patients (26.6%) had skeletal manifestations of Marfan's syndrome. Additional cardiac procedures were performed in two patients (13.4%). Mean follow-up time was 26.6+/-11.2 months (range 7-50 months). RESULTS: There were no early deaths. Postoperative non-fatal complications occurred in two (13.3%) patients. There was no valve-related late death occurring 6 months after operation. Actuarial survival was 0.94+/-0.07 at 4 years; 4-year freedom from reoperation was 0.91+/-0.07. The probability of needing reoperation was 8+/-0.9%. There were no instances of infective endocarditis or thromboembolic complications. At the end of the follow-up period, trivial or no aortic regurgitation was demonstrated in 57.1%, mild in 42.9% and no patient had an insufficiency graded >/=moderate. Echocardiography showed early postoperative reduction in left ventricular end-systolic and end-diastolic dimensions, which was maintained at follow-up controls. CONCLUSIONS: The Tirone David I procedure showed good early results, which encourage further use of this type of repair. However, further larger studies and long-term results are necessary for a definite judgment on this operative method.

A long-term experience with the Cabrol root replacement technique for the management of ascending aortic aneurysms and dissections.

BACKGROUND: Little information exists regarding the long-term results of the Cabrol technique. This study aimed at exploring, over a 16-year period, the results of aortic root replacements employing this procedure. METHODS: Between 1986 and 2002 forty-five patients (mean age 58.7 +/- 13.8 years old, 84.4% male) underwent a Cabrol procedure. Aortic dissection was the most frequent cause of aortic disease in this series (n = 17), followed by annuloaortic ectasia (n = 10), atherosclerotic aneurysm (n = 5) and poststenotic dilatation (n = 5). Six patients (13.4%) had undergone a previous aortic operation, 8 (17.7%) had a Marfan syndrome and five (11.1%) underwent concomitant arch replacement. Mean clinical follow-up was 87.3 +/- 24.3 months. Twenty-eight patients (93.3% of survivors) had a transesophageal echocardiography (TEE) performed at a mean of 64 +/- 32 months postoperatively. RESULTS: Early mortality was 20%. It was 9.1% for patients with an ascending aortic aneurysm and 41.2% for dissections (p = 0.026). Independent multivariate predictors of early mortality were: aortic dissection (p = 0.009), emergency operation (p < 0.001), operative year (p = 0.02), cross-clamp time (p = 0.001), and CPB duration (p < 0.001). Actuarial survival was 0.77 +/- 0.06, 0.72 +/- 0.06, 0.59 +/- 0.04 and 0.59 +/- 0.04 at 1, 5, 10, and 16 years, respectively. Multivariate analysis revealed age (p = 0.007), cross-clamp time (p = 0.0006) and CPB duration (p = 0.009) as strong predictors of poor late survival. A periprosthetic jet with significant valve regurgitation was detected by TEE in 3 patients. In one of them, an infected periprosthetic space-right ventricular fistula was demonstrated requiring reoperation. Altogether, freedom from reoperation and endocarditis at 16 years was 0.97 +/- 0.02 and 0.94 +/- 0.03, respectively. CONCLUSIONS: The Cabrol technique demonstrated a nonnegligible incidence of early and long-term complications. It should be rarely used and only when a "button" technique is not feasible.

Functional evaluation of steal by unligated first intercostal branch before transcatheter embolization in recurrent angina after a LIMA-LAD graft.

Unligated side branches of the left internal mammary artery (LIMA) have been indicated as a cause of coronary steal resulting in postoperative angina. Although a number of studies have reported successful embolization of the side branches to relieve angina, this phenomenon is still controversial and it has been either emphasized or rejected in studies that attempted to obtain hemodynamic evidence of the steal using angiographic and intravascular Doppler techniques. In this case study, we tried to reproduce physiological decrease in the muscular and coronary beds as it could occur during activity. Our results, using an intracoronary Doppler wire with adenosine combined with forced ventilation, showed that a trial occlusion of the LIMA side branch with a balloon technique can demonstrate whether the flow through the LIMA would increase after embolization of the side branch.

Video-assisted thoracoscopic surgery for primary chylopericardium after orthotopic heart transplantation.

Chylopericardium is a rare complication of cardiac surgical procedures. We describe a 41-year-old woman who underwent orthotopic heart transplantation and developed chylopericardium early in the post-operative period. Because of unsuccessful conservative treatment (pericardiocentesis, pericardial drainage, low-fat diet with medium-chain triglycerides, and total parenteral nutrition), surgical intervention was indicated and the patient successfully underwent right-sided thoracoscopic duct ligation and partial pericardiectomy (pericardial window).

Preliminary experience with the St. Jude Medical Regent mechanical heart valve in the aortic position: early in vivo hemodynamic results.

BACKGROUND: The St. Jude Medical Regent is a new generation mechanical aortic valve. METHODS: Between March 2000 and July 2001, this valve was implanted in the aortic position in 40 patients (21 men; mean age 59.1 +/- 9.0 years). Preoperatively, 24 patients (60%) were in New York Heart Association functional class III or IV. Eighteen patients (45%) underwent associated procedures. Mean valve size was 21.4 +/- 2.4 mm. The mean duration of follow-up was 8.5 +/- 4.5 months (range, 1 to 16 months). RESULTS: There were no operative deaths. Early complications included one reoperation for bleeding and one transient low output syndrome. Valve replacement was followed by a significant reduction in mean and peak transaortic gradients over time (p < 0.001) and analysis of variance failed to demonstrate statistical differences between valve size over time (p = not significant). A significant reduction in left ventricular hypertrophy occurred over time (p = 0.01) in all valve sizes (p = not significant between groups): baseline left ventricular mass index was 194 g/cm2; it reduced by 22 g/cm2 (p = 0.006) at discharge. Left ventricular mass index decreased from 172 +/- 55 g/cm2 to 156 +/- 44 g/cm2 (p = 0.03) from discharge to 2 months. Further reductions were not significant. Relative wall thickness decreased from 0.57 +/- 0.13 preoperatively to 0.42 +/- 0.06 at discharge (p = 0.001), and again at 2 months (-0.2; p = not significant), and at 1 year (-0.02; p = not significant). CONCLUSIONS: The early experience with the St. Jude Medical Regent valve has been satisfactory.