Right ventricular cardiomyopathies: a multidisciplinary approach to diagnosis.

The physiological importance of the right ventricle (RV) has been underestimated over the past years. Finally in the early 1950s through the 1970s, cardiac surgeons recognized the importance of RV function. Since then, the importance of RV function has been recognized in many acquired cardiac heart disease. RV can be mainly or together with left ventricle (LV) affected by inherited or acquired cardiomyopathy. In fact, RV morphological and functional remodeling occurs more common during cardiomyopathies than in ischemic cardiomyopathies and more closely parallels LV dysfunction. Moreover, there are some cardiomyopathy subtypes showing a predominant or exclusive involvement of the RV, and they are probably less known by cardiologists. The clinical approach to right ventricular cardiomyopathies is often challenging. Imaging is the first step to raise the suspicion and to guide the diagnostic process. In the differential diagnosis, cardiologists should consider athlete's heart, congenital heart diseases, multisystemic disorders, and inherited arrhythmias. However, a multiparametric and multidisciplinary approach, involving cardiologists, experts in imaging, geneticists, and pathologists with a specific expertise in these heart muscle disorders is required.

Far field R-wave sensing in Myotonic Dystrophy type 1: right atrial appendage versus Bachmann's bundle region lead placement.

Aim of the present study was to investigate far field R-wave sensing (FFRS) timing and characteristics in 34 Myotonic Dystrophy type 1 (DM1) patients undergoing dual chamber pacemaker implantation, comparing Bachmann's bundle (BB) stimulation (16 patients) site with the conventional right atrial appendage (RAA) pacing site (18 patients). All measurements were done during sinus rhythm and in supine position, with unipolar (UP) and bipolar (BP) sensing configuration. The presence, amplitude threshold (FFRS trsh) and FFRS timing were determined. There were no differences between both atrial sites in the Pmin and Pmean values of sensed P-wave amplitudes, as well as between UP and BP sensing configurations. The FFRS trsh was lower at the BB region in comparison to the RAA site. The mean BP FFRS trsh was significantly lower than UP configuration in both atrial locations. There were no significant differences in atrial pacing threshold, sensing threshold and atrial lead impedances at the implant time and at FFRS measurements. Bachmann's bundle area is an optimal atrial lead position for signal sensing as well as conventional RAA, but it offers the advantage of reducing the oversensing of R-wave on the atrial lead, thus improving functioning of standard dual chamber pacemakers in DM1 patients.

Impact of obesity on left ventricular geometry and function in pediatric patients after successful aortic coarctation repair.

AIMS: To evaluate if obesity has an additional negative impact on left ventricular (LV) geometry and function in normotensive pediatric patients >12 months after successful treatment of aortic coarctation (CoA). METHODS AND RESULTS: We studied 40 CoA patients (mean age 14 ± 3 years, and male sex 70%), of them 10 were obese and 30 lean. Both groups were age and sex comparable. The entire studied sample underwent 24-ambulatory blood pressure (BP) monitoring, standard echocardiographic evaluation, and speckle tracking study. Both office and 24-hour diastolic BP were significantly increased in obese patients. Obese CoA patients showed increased LV mass (52 ± 13 g/m(2.7) vs. 43 ± 9 g/m(2.7) , P = 0.02), and significant reduction in E/A compared with lean CoA patients. Myocardial deformation properties were significantly reduced in obese CoA patients in all the three studied planes (longitudinal, radial, and circumferential) compared with CoA lean patients. LV twist values showed a significant reduction in the obese CoA group (9.9° ± 2.2° vs. 14.5° ± 2.3°, P < 0.0001). CONCLUSIONS: Our study shows that obesity in successfully treated CoA children, has an additional negative effect on BP, LV mass, and cardiac function. These findings are of particular concern, since life expectancy in CoA patients is limited mainly by atherosclerosis, and all the obesity-associated abnormalities found are harbingers of higher cardiovascular risk.

Abnormal regional myocardial deformation properties and increased aortic stiffness in normotensive patients with aortic coarctation despite successful correction: an ABPM, standard echocardiography and strain rate imaging study.

The long-term follow-up data subsequent to a successful repair of AoC (aortic coarctation) show that life expectancy remains reduced. Previous standard echocardiographic studies have demonstrated normal or increased systolic cardiac function in patients following successful repair of AoC. SR (strain rate) imaging is a new technique able to detect subclinical myocardial abnormalities. In the present study we investigated whether young patients (without hypertension, as assessed using ambulatory blood pressure monitoring and an exercise test) following successful AoC repair already have abnormal myocardial deformation properties, and the relationship of the deformation properties with aortic stiffness. We studied 166 subjects, 83 AoC non-hypertensive patients (mean age 12+/-4 years) a number of years after successful repair of AoC and 83 age- and sex-matched subjects as controls. Peak systolic SR (1/s) for both regional longitudinal and radial function was assessed. The aortic stiffness index was calculated from the echocardiographically derived thoracic aortic diameters, and the measurement of blood pressure was obtained by cuff sphygmomanometry. The LV (left ventricular) ejection fraction was significantly increased in AoC patients, whereas regional longitudinal SRs were significantly reduced (-1.1+/-0.9 compared with -2+/-0.5, P<0.0001) in patients. The aortic stiffness index was significantly increased in AoC patients (12+/-9, P<0.0001). At multilinear regression analysis, age at repair (P=0.005; coefficient, -0.201; S.E.M., 0.027) and the aortic stiffness index (P=0.0029; coefficient, 0.334; S.E.M., 0.423) predicted longitudinal SR. Despite the presence of a successful repair for AoC, in the absence of hypertension, longitudinal deformation properties were significantly impaired. Moreover, the degree of longitudinal SR impairment was correlated with age at repair and aortic stiffness. Early repair can delay the onset of hypertension in postcoarctectomy patients, but cannot prevent the innate structural and functional abnormalities of the aorta and their deleterious effect on myocardial deformation properties.

The impact of age and gender on right ventricular diastolic function among healthy adults.

BACKGROUND: Doppler echocardiography is ideally suited for assessment of diastolic function, being widely available, non-invasive, and less expensive than other techniques. However, data regarding age- and gender-matched reference values of right ventricular diastolic function are limited. This study aims to explore the physiologic variations of right ventricle (RV) diastolic function in a large cohort of healthy adults, and to investigate clinical and echocardiographic correlates. METHODS: From June 2007 to February 2014, 1168 healthy Caucasian subjects [mean age 45.1±15.6 years, range 16-92; 555 (47.5%) men] underwent comprehensive transthoracic echocardiography (TTE) following current guidelines. The following RV main diastolic measurements were measured: peak early inflow velocity (E), annular both early (e') and atrial (a') velocities, E/e' ratio. RESULTS: RV E/e' constantly increases with age in females, but do not change substantially in males. RV E/A constantly decreases with age in both genders. Stepwise multiple linear regression analysis underlined a close significant association of RV diastolic function with both right and left heart morphologic measurements (right atrial area, RV diameters, left atrial volume) and functional indexes (TAPSE, RV tissue Doppler peak systolic velocity, left ventricular E/Ee'), as well as with indexes of increased pulmonary resistance. CONCLUSION: Our data highlight the potential usefulness of different normal reference values according to the age and gender to correctly evaluate RV diastolic function. Differences in terms of demographic and anthropometric parameters could be useful to avoid potential misclassification of RV diastolic function when based on dichotomously suggested normal cut-off values.

Strain rate imaging is a superior method for the assessment of regional myocardial function compared with Doppler tissue imaging: a study on patients with transcatheter device closure of atrial septal defect.

To compare the value of strain and strain rate imaging in assessing regional myocardial function to that of myocardial velocities, we studied 10 patients late after successful percutaneous atrial septal defect closure. Analysis was performed for atrial longitudinal peak systolic velocity, strain, and strain rate from the midsegment of interatrial septum, in correspondence of the device, and from the lateral left atrial wall. Placing the sample volume in the middle part of the atrial septal defect occluder, a bulky noncontractile element, passively moved by global heart motion, the new ultrasonic-derived myocardial deformation indexes demonstrated almost the absence of any deformations, whereas myocardial velocities failed to significantly discriminate between this noncontracting structure and the normal atrial wall.

Electrophysiological changes following balloon valvuloplasty and angioplasty for aortic stenosis and coartaction of aorta: clinical evidence for mechano-electrical feedback in humans.

BACKGROUND: Basic research and animal experiments have shown electrophysiological changes during or after changes in mechanical loading. Electrical instability following mechanical stretch has been observed as development of after-depolarisation and dispersion of refractoriness and repolarisation. The aim of the present study was to evaluate the presence of the mechano-electrical feedback in humans, assessing the ventricular repolarisation changes following acute changes in left ventricular pressure. MATERIAL AND METHODS: The study group comprised 30 consecutive patients (22 M and 8 F, aged 2 days-24 years) affected by severe congenital aortic stenosis and 30 patients (20 M and 10 F, aged 6 months-16 years) affected by severe coartaction of aorta. Ventricular repolarisation was evaluated before and after percutaneous balloon valvuloplasty and angioplasty in terms of absolute measures (JT, JTc, QT, QTc) and in terms of dispersion across the myocardium: QT and QTc dispersion (QTD, QTcD), JT and JTc dispersion (JTD and JTcD) and T-peak to T-end interval (Tp-Te). RESULTS: Patients with severe aortic stenosis and patients with aortic coartaction showed a significant decrease in dispersion of ventricular repolarisation time indexes (QTD, QTcD, JTD, JTcD and Tp-Te) following valvuloplasty and angioplasty. CONCLUSIONS: Changes in hemodynamic loading can also produce electrophysiological effects in humans. Acute reduction in left ventricular pressure overload following balloon valvuloplasty and angioplasty, decreases electrical instability, as expressed by the reduction across the myocardium of the dispersion of ventricular repolarisation.

Tissue Doppler imaging in systemic sclerosis: a 3-year longitudinal study.

OBJECTIVES: To investigate by standard echocardiography and pulsed-tissue Doppler imaging (TDI) the course of systemic sclerosis (SSc) heart disease and its correlation with epidemiological, clinical, and serological features of the disease and drug treatment. METHODS: A total of 74 consecutive patients (69 females, between the ages of 19 and 71 years, and disease duration 1-43 years) and 71 controls underwent cardiac assessment at baseline and at 3-year follow-up. RESULTS: At baseline, compared to controls, patients showed post-Bonferroni correction, impaired left (LV) and right ventricular (RV) diastolic function (Em/Am 0.85 ± 0.4 vs 1.5 ± 0.7, p = 0.0003; Et/At 0.9 ± 0.3 vs 1.3 ± 0.4, p = 0.0003), subtle LV and RV systolic dysfunction (Sm 13.7 ± 2.7 vs 15.4 ± 3.2cm/s, p = 0.031; St < 11.5cm/s in 16/74 patients vs 0 controls, p = 0.0031), and higher pulmonary artery systolic pressure (sPAP) (26.1 ± 6.0 vs 24.1 ± 5.1, p = 0.040). At 3-year follow-up, SSc patients showed a further deterioration of biventricular diastolic and systolic function and a further sPAP increase. At multiple regression analysis of baseline data, Em/Am < 1 was detected in 55/74 patients vs 25/71 controls (p < 0.0001) and was associated with age (p = 0.030); Et/At < 1 was detected in 16/74 patients vs 7/71 controls (p < 0.0001), was associated with NYHA class ≥ II (p = 0.033), late capillaroscopic pattern (p = 0.029), and a baseline cardiac Medsger severity score ≥ 1 (p = 0.029). TDI evidence of new abnormalities in RV and/or LV diastolic function was associated with a baseline cardiac Medsger severity score ≥ 1 (p = 0.01). Neither diastolic or systolic abnormalities nor sPAP changes correlated with treatment. CONCLUSIONS: Our study confirms that SSc patients exhibit biventricular systolic and diastolic dysfunction and increased sPAP and reveals further deterioration at 3-year follow-up.

Stress stent fracture: is stent angioplasty really a safe therapeutic option in native aortic coarctation?

An 11-year-old boy was successfully treated by stent implantation for native aortic coarctation. At the 1-year control a severe re-coarctation was found at Doppler analysis and subsequent angiography revealed a transverse stent fracture. A stent-in-stent implantation was performed. Several hypotheses could explain this complication. Interventional cardiologists may pay more attention in following up these patients!