Right cardiac involvement in lung diseases: a multimodality approach from diagnosis to prognostication.

Lung diseases are amongst the main healthcare issues in the general population, having a high burden of morbidity and mortality. The cardiovascular system has a key role in patients affected by respiratory disorders. More specifically, the right ventricle (RV) enables the impaired lung function to be overcome in an initial stage of disease process, reducing the severity of dyspnoea. In addition, two of the main causes of death in this setting are RV failure and sudden cardiac death (SCD). Echocardiography is regarded as a useful and easily available tool in assessing RV function. Several noninvasive echocardiographic parameters of elevated pulmonary pressures and RV function have been proposed. The combination of different parameters and imaging methods is paramount and researches regarding RV impairment using these indices has been specifically addressed in relation to the chronic obstructive and restrictive lung disease in order to guide the clinicians in the management of these patients. Cardiac involvement in lung diseases is often observed, and RV changes are reported also in early stages of pulmonary diseases. The role of right ventricle in chronic respiratory disease patients has to be evaluated in detail to describe the response to therapy and the degree of disease progression through multimodality and advanced imaging techniques. The aim of this review is to describe the different pathophysiological mechanisms of cardiac impairment in primary lung disease (such as chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF) and sarcoidosis) and to summarize the role of cardiac multimodality imaging in the diagnosis and the prognosis of these diseases.

Case Report: Aortic Valve Replacement After JARVIK 2000 Left Ventricular Assist Device Implantation in Long-Time Survivor With Severe Aortic Valve Regurgitation.

BACKGROUND: There are limited clinical records in the literature regarding aortic valve replacement in left ventricular assist device (L-VAD) patients. Previously we had two cases of severe aortic valve regurgitation in patients with L-VAD support treated with Corvalve prosthesis insertion and Amplatzer closure procedure. Both patients died a few days after the procedure from complications not related to the procedure itself. PATIENT HISTORY: The patient was a male with previous coronary artery bypass graft surgery in 2001 that was complicated with postischemic dilated cardiomyopathy with severe heart failure (ejection fraction [EF], 20%). Cardiac resynchronization therapy was biventricular-pacemaker and cardiac defibrillator implantation in 2009 for recurrent ventricular arrhythmia. L-VAD implantation (Jarvik 2000) with graft apposition in descending thoracic aorta through left thoracotomy access and retro-auricolar cable was performed in October 2013. In 2015 the patient underwent surgical aortic valve replacement with bioprothesis due to progressive worsening of the aortic valve regurgitation. The Jarvik 2000 outflow was occluded with vascular ball occluder inserted via right axillary artery under fluoroscopy before CEC installation. The recovery was without major complications. DISCUSSION: Long-time survivors with Jarvik 2000 are increasing in number and such late complication is expected to become a main future issue. Our previous experience with the interventional approach was delusive. Due to the fatal consequences in similar patients with nonsurgical approaches, we opted for surgical aortic valve replacement. At the moment, the international literature does not describe safe approaches regarding aortic valve replacement in patients with Jarvik 2000 L-VAD. This case shows that surgical valve replacement could be managed with success according to the described specific technique.

First Evidence of Cardiac Stem Cells From the Left Ventricular Apical Tip in Patients With Left Ventricular Assist Device Implantation.

BACKGROUND: Recent studies have challenged the dogma that the adult heart is a postmitotic organ and raise the possibility of the existence of resident cardiac stem cells (CSCs). Our study aimed to explore if these CSCs are present in the "ventricular tip" obtained during left ventricular assist device (LVAD) implantation from patients with end-stage heart failure (HF) and the relationship with LV dysfunctional area extent. METHODS: Four consecutive patients with ischemic cardiomyopathy and end-stage HF submitted to LVAD implantation were studied. The explanted "ventricular tip" was used as a sample of apical myocardial tissue for the pathological examination. Patients underwent clinical and echocardiographic examination, both standard transthoracic echocardiography (TTE) and speckle tracking echocardiography (STE), before LVAD implantation. RESULTS: All patients presented severe apical dysfunction, with apical akinesis/diskinesis and very low levels of apical longitudinal strain (-3.5 ± 2.9%). Despite this, the presence of CSCs was demonstrated in pathological myocardial samples of "ventricular tip" in all 4 of the patients. It was found to be a mean of 6 c-kit cells in 10 fields magnification 40×. CONCLUSIONS: Cardiac stem cells can be identified in the LV apical segment of patients who have undergone LVAD implantation despite LV apical fibrosis.

A quantitative assessment of circulating progenitor cells in competitive athletes and in sedentary subjects.

AIM: Hematopoietic stem cells (HSCs) and endothelial progenitor cells (EPCs) are known to play a role in the vascular responses and adaptations to exercise. We performed a quantitative assessment of HSCs and EPCs in adolescents in order to investigate whether resting levels of circulating HSCs and EPCs are comparable between elite athletes and sedentary healthy subjects. METHODS: HSCs and EPCs levels were measured in adolescent competitive football players and in age- and sex-matched sedentary controls. A laboratory testing was also performed to determine the white blood cells count and the lipid profile. All athletes were evaluated at the same stage of their training program, after 6 months of training. Controls were not engaged in any kind of routine training program. RESULTS: Twenty male competitive athletes (18.4 ± 0.5 years) and 9 sedentary controls (18.7 ± 0.4 years) participated in the study. As expected, HDL cholesterol was higher in athletes as compared with controls (P<0.05). No significant differences in the other laboratory parameters were observed among groups. Circulating levels of HSCs were significantly lower in athletes in comparison with sedentary controls (P<0.05). Conversely, EPCs and KDR+ cell subpopulations did not substantially differ between athletes and controls. CONCLUSION: Adolescent athletes exhibit lower levels of circulating HSCs but not of EPCs compared to sedentary controls. The process of tissue repair associated with intensive training can contribute to this difference, acting as a stimulus for mobilization and homing of HSCs in the site of injuries.

The role of 3D and speckle tracking echocardiography in cardiac amyloidosis: a case report.

Cardiac amyloidosis (CA) is a disorder characterized by amyloid fibrils deposition in cardiac interstitium; it results in a restrictive cardiomyopathy with heart failure (HF) and conduction abnormalities. The "gold standard" for diagnosis of CA is myocardial biopsy but possible sampling errors and procedural risks, limit it's use. Magnetic resonance (RMN) offers more information than traditional echocardiography and allows diagnosis of CA but often it's impossible to perform. We report the case of a man with HF and symptomatic bradyarrhythmia that required an urgent pacemaker implant. Echocardiography was strongly suggestive of CA but wasn't impossible to perform an RMN to confirm this hypothesis because the patient was implanted with a definitive pacemaker. So was performed a Speckle Tracking Echocardiography (STE) and a 3D echocardiography: STE allows to differentiate CA from others hypertrophic cardiomyopathy by longitudinal strain value < 12% and 3D echocardiography shows regional left ventricular dyssynchrony with a characteristic temporal pattern of dispersion of regional volume systolic change. On the basis of these results, finally was performed an endomyocardial biopsy that confirmed the diagnosis of CA. This case underlines the importance of news, noninvasive techniques such as eco 3D and STE for early diagnosis of CA, especially when RMN cannot be performed.

Severity of aortic stenosis predicts early post-operative normalization of left atrial size and function detected by myocardial strain.

BACKGROUND: Aortic stenosis (AS) causes significant disturbances in left ventricular (LV) and left atrial (LA) function irrespective of the extent of myocardial hypertrophy which associates the increased afterload. We hypothesize that aortic valve replacement (AVR) and removal of LV outflow tract obstruction should result in LA size and function recovery, even partial, and were set to study this in a group of patients with AVR for AS. METHODS: Peak atrial longitudinal strain (PALS) was evaluated in 43 patients with severe isolated AS and normal EF (56.6 ± 3.8%) and no obstructive coronary artery disease candidates for AVR, pre-operatively and then 40 days and 3 months after surgery. Results were compared with those from 34 age- and gender-matched healthy controls. RESULTS: LVEF remained unchanged and LV mass regressed after AVR. Global PALS was reduced pre-operatively and increased 40 days after surgery (p=0.002) and showed only a slight further increase at 3 months follow-up (p<0.0001). Indexed LA volume was increased before surgery, but significantly fell 40 days after surgery (p<0.0001) and showed only a slight further reduction after 3 months (p<0.0001). Trans-aortic mean gradient change after surgery was the only independent predictor of the recovery of LA size and function. CONCLUSIONS: AVR reverses LA abnormalities and regains normal atrial function, a behavior which is directly related to the severity of pre-operative LV outflow tract obstruction. Early identification of LA size enlargement and functional disturbances might contribute to better patient's recruitment for AVR.

Mitral annular longitudinal function preservation after mitral valve repair: the MARTE study.

BACKGROUND: In patients with chronic mitral regurgitation (MR), undergoing surgical mitral valve repair, current Guidelines only recommend standard echocardiographic indices i.e. left ventricular (LV) ejection fraction (EF), and LV end-systolic and end-diastolic diameters as preoperative variables. However LV EF is often preserved until advanced stages of the valve disease. Aim of this study was to evaluate changes in LV systolic longitudinal function, 3 months after mitral valve repair in patients with chronic degenerative MR and normal preoperative EF. METHODS: We measured M-mode mitral lateral annulus systolic excursion (MAPSE) and Tissue Doppler (TD) peak systolic annular velocity (S(m)) in 31 patients with moderate to severe MR and normal EF (59.9 ± 4.7%) candidates for mitral valve repair, preoperatively and 3 months after surgery. RESULTS: After mitral valve repair, S(m) increased from 7.8 ± 1.4 to 9.6 ± 2.2 cm/s (p<0.0001) and MAPSE increased from 1.33 ± 0.26 to 1.55 ± 0.25 cm (p=0.0013). EF decreased from 59.9 ± 4.7 to 51.3 ± 5.9% (p<0.0001). As expected, LV diameters and volumes, wall thicknesses, midwall fractional shortening (mFS), and left atrial (LA) size were all reduced after surgery. CONCLUSIONS: This study suggests that assessment of LV long axis systolic velocity and amplitude of excursion by echocardiography is more sensitive than simple determination of EF for revealing the beneficial impact of MR surgery on overall systolic function.

New onset of electrocardiographic abnormalities heralding hypertrophic cardiomyopathy in an adult athlete.

A 41-year-old male asymptomatic athlete with unremarkable personal and family history of heart disease underwent annual preparticipation screening. No abnormalities were noted on prior testing. On this occasion, a 12-lead electrocardiogram showed diffused and marked repolarization abnormalities. He was therefore referred for echocardiography, which showed moderate asymmetric hypertrophy localized at the mid-apical portions of the left ventricular anterolateral wall. Cardiac magnetic resonance confirmed the diagnosis of hypertrophic cardiomyopathy. Re-evaluation of the electrocardiogram performed the previous year revealed a completely normal tracing.

Surgical treatment of abdominal aortic aneurysms associated with aortic valve incompetence: strategies and outcomes.

BACKGROUND: The exact incidence of associated aortic valve incompetence (AVI) and abdominal aortic aneurysm (AAA) in the general population is not known. In recent years, we have observed this association with increasing frequency. This observation is probably due to the extensive preoperative screening of the cardiac and vascular status of patients who are candidates for surgical procedures. The choice of the optimal surgical strategy is needed to achieve low operative morbidity and mortality. The present study reviews our experience with a subset of patients suffering the association of AVI and large AAA. Surgical strategy, clinical management and outcome are presented. METHODS: Between January 1982 and May 2000, 76 patients with the association of AAA and AVI have been evaluated in our institution. Forty-four patients have been treated for both AAA and aortic valve (AV) regurgitation. These patients have been divided into three groups on the basis of the surgical strategy adopted. Group 1: combined procedure (16 patients); group 2: AAA repair prior to AV surgery (nine patients); group 3: AV surgery prior to aneurysm repair (19 patients). RESULTS: Hospital mortality was 4.5% (two patients); overall mortality was 6.8% (three patients). CONCLUSIONS: In patients with AAA and AVI, an accurate and complete preoperative evaluation is essential. Surgical strategy should be individualized on the basis of the cardiac preoperative status.

Noninvasive tests for risk stratification in major vascular surgery.

BACKGROUND: The predictive values of noninvasive tests versus perioperative cardiac events in patients undergoing major vascular surgery has not been definitively established. PATIENTS AND METHODS: According to clinical markers and left ventricular function at rest, 188 patients were assigned to the following groups: 40 low, 115 moderate and 33 high risk. They were then randomly submitted to dipyridamole (n = 64), dobutamine (n = 63) stress echocardiography and dipyridamole perfusion scintigraphy (n = 61). RESULTS: No events were observed in low-risk patients, whereas 12 (10.4%) and 8 (24%) events in moderate- and high-risk categories occurred, respectively. Only the high-risk category, as a predictive variable, was significantly related to the onset of cardiac complications (p < 0.05). A positive dipyridamole/dobutamine stress test was related to cardiac events, but multivariate analysis showed that only severity and extent of ischemia were the best predictors of events (p < 0.01 for dipyridamole and p < 0.005 for dobutamine). The presence of reversible, but not fixed, perfusion defects at scintigraphy was significantly related to perioperative events; at multivariate analysis, only > 3 reversible perfusion defects represented a strong predictor of events (p < 0.05). CONCLUSIONS: Among subjects undergoing major vascular surgery, severity and extent of ischemia during dipyridamole/dobutamine stress echocardiography and presence of > 3 reversible perfusion defects are strong predictors of cardiac events, particularly in moderate-risk category of patients.

A primary intracavitary right atrial neurilemoma.

Primary cardiac neurilemoma, a benign tumor, is extremely uncommon. To our knowledge only eight cases have been reported in the literature. We report a case of a 72-year-old man who presented with complaints of progressive shortness of breath and chest pain, seven years after a right nephrectomy for renal adenocarcinoma. An intra-right atrial tumor was surgically removed; the lesion was found to be a neurilemoma of the right atrium. This case report describes the surgical removal and rarity of neurilemomas, their predisposition to be right-sided in the heart and their coincidental association with other types of cancer.

Resting ECG is modified after oophorectomy and regresses with estrogen replacement therapy in premenopausal women.

OBJECTIVE: To assess the effects of bilateral oophorectomy on the resting ECG and whether they regress with estrogen replacement therapy. STUDY DESIGN: Twenty-six premenopausal and 15 postmenopausal women were enrolled in the present study. All women had undergone hysterectomy and bilateral ovariectomy. All women underwent 12-lead ECG on admission to hospital. A second ECG was recorded 20-25 days after surgery. After this second ECG, premenopausal women were randomly divided into two groups. The women of Group A (n=14) received transdermal ethinyl estradiol (EE). The women of Group B (n=12) did not receive any therapy. A third ECG was performed in both groups 30-35 days after randomization. RESULTS: Bilateral oophorectomy did not induce any significant modifications in the ECG parameters of the postmenopausal women whereas in the premenopausal women, we observed a significant increment in mean duration of the T wave, a significant decrease in its amplitude and significant reduction in ST depression in V2, V3, V4 and V5. The third ECG showed regression of the ECG modifications in Group A. In the women of Group B, the second and third ECGs were not substantially different, but there were statistically significant differences between the first and third ECGs. CONCLUSIONS: The results of the present study show that ovariectomy induces significant though not clinically evident modifications in resting ECG. These ECG changes are probably due to the sudden reduction in sex hormone plasma levels after ovariectomy. Administration of estradiol induced regression of the ECG modifications.

Cerebrotendinous xanthomatosis: evidence of lipomatous hypertrophy of the atrial septum.

Premature atherosclerosis and cardiac complications have been reported among the systemic manifestations of cerebrotendinous xanthomatosis (CTX), a rare bile acid disorder with predominantly neurological features. In some cases, myocardial infarction has been the cause of sudden death. We examined nine CTX patients to determine whether they also had clinical or subclinical signs of cardiovascular disorders. In four of them, transthoracic echocardiography showed thickening of the interatrial septum compatible with lipomatous hypertrophy. The unexpected association of the two abnormalities is unlikely to be coincidental and suggests that careful cardiac examination should be considered, even in the absence of clinical manifestations.

Reactive arthritis by staphylococcus epidermidis: report of an unusual case.

In the literature many cases of Staphylococcus epidermidis (SE) complications are reported, but we have not found any reference about reactive arthritis secondary to SE. We report an unusual case of a patient with SE bacteriaemia, who developed elbow arthritis, asymmetrical sacroiliitis, keratoderma and restrictive cardiomyopathy. The clinical pictures, the instrumental and biochemical findings, in particular the positivity of HLA B27, allow us to set this case in the complex and heterogeneous chapter of reactive arthritis.

Comparison between a cisplatin-containing regimen and a carboplatin-containing regimen for recurrent or metastatic bladder cancer patients. A randomized phase II study.

BACKGROUND: The aim of this randomized Phase II study was to compare the efficacy and toxicity of a cisplatin-containing regimen with a carboplatin-containing regimen for patients with recurrent or metastatic bladder cancer. METHODS: Fifty-seven patients with recurrent or metastatic bladder cancer were randomized to receive M-VEC treatment (methotrexate, vinblastine, epirubicin, and cisplatin) (n = 29) or M-VECa treatment (methotrexate, vinblastine, epirubicin, and carboplatin) (n = 28). The chemotherapy was scheduled at 28-day intervals. Recombinant granulocyte-colony stimulating factors were administered daily when the absolute neutrophil count fell below 1000/mm3. The development of ototoxicity was evaluated by measuring auditory brain stem response. RESULTS: Of the 57 entered patients, 55 were evaluable for response and toxicity. The overall clinical response rate was 71% (with 25% complete responses) in the M-VEC group and 41% (with 11% complete responses) in the M-VECa group (P = 0.04). M-VEC chemotherapy was associated with more pronounced side effects. There was a statistically significant difference between M-VEC and M-VECa in terms of gastrointestinal toxicity (P = 0.04), nephrotoxicity (P = 0.03), and neurotoxicity (P = 0.02) during Cycle 3 of chemotherapy. Leukopenia and neutropenia were worse in the M-VECa arm, but not significantly so (P = 0.4). Ototoxicity was only detected in one of seven examined M-VEC patients after two cycles of chemotherapy. CONCLUSIONS: M-VECa has a low level of gastrointestinal, renal, neurologic, and otologic toxicity, but is apparently less effective than M-VEC in the treatment of recurrent or metastatic bladder cancer. However, a larger, randomized Phase III trial is needed to confirm these results.

Weekly chemotherapy in advanced prostatic cancer.

This randomised phase II study was performed in order to evaluate the effectiveness of a weekly chemotherapy regimen in advanced prostatic carcinoma patients (stage D2) refractory to hormonal therapy. Seventy-two cases were studied: they were randomised in a 2:1 ratio to receive either epirubicin (30 mg m-2 weekly) or doxorubicin (25 mg m-2 weekly); 48 patients received epirubicin and 24 received doxorubicin. After 12 courses of chemotherapy, the 45 evaluable patients in the epirubicin arm showed a response rate of 37.7% and the 21 evaluable patients in the doxorubicin arm showed a response rate of 33.3% (P = 0.51). Pain intensity, bone and prostatic tumour markers rapidly and significantly decreased in responders. An improvement in physical symptoms, functional conditions and in emotional well-being was observed in the majority of the treated patients. The histological analysis of bone metastases, performed before and after 12 courses of chemotherapy showed a significant reduction in neoplastic invasion and in new bone formation in responders. Cardiac performance worsened in five out of 45 patients and in ten out of 21 during the first 12 courses of epirubicin or doxorubicin respectively (P = 0.014). The median survival was 12.5 months in the epirubicin arm and 8.0 months in the doxorubicin arm (P = 0.042). Our data indicate that in advanced prostatic carcinoma, a weekly epirubicin regimen may give rapid palliative results, similar to that of doxorubicin, but with less side-effects.

[Echocardiographic diagnosis of left ventricular myxoma. Description of a case and review of the literature. Myxomas of the left ventricle]. / Diagnostica ecocardiografica del mixoma ventricolare sinistro. Descrizione di un caso e revisione della letteratura. I mixomi del ventricolo sinistro.

A case of left ventricular myxoma diagnosed by echocardiography and successfully removed by left ventriculotomy is reported. This is a 21 year old male, with the few symptoms which simulating an hypertrophic cardiomyopathy in contrast to the large size of the tumour. It is possible that myxomas are responsible for sudden death. Therefore, in presence of new cardiac signs kind and relevance, the possibility of a myxoma should be considered. The diagnosis can be easily ruled out (or confirmed) by echocardiography, which represents a valuable tool in the diagnosis of myxomas.