Rachele Adorisio

Pharmacological treatment of chronic heart failure

Heart failure (HF) is associated with a high morbidity and mortality in the Western World. Our knowledge of the epidemiology, pathophysiology, and therapy has improved dramatically during the last 20 years. Pharmacological treatment, as it stands today, is a combination of preventive and symptomatic strategies. The mainstay life-saving drugs are angiotensin-converting enzyme inhibitors and β-blockers. Additional benefits are obtained when angiotensin-receptor blockers or aldosterone antagonists are added. Digitalis and/or diuretics are useful for symptom reduction. In addition, combination therapy with hydralazine and isosorbide dinitrate is recommended in African Americans.

Plastic Bronchitis After Extracardiac Fontan Operation

BACKGROUND We discuss 4 cases of plastic bronchitis (PB) after Fontan procedure observed at our Institution, with the aim to identify an effective treatment and possible factors favoring the onset of PB. Plastic bronchitis is an uncommon disease characterized by recurrent formation of large pale bronchial casts obstructing the tracheobronchial tree. The treatment includes inhaled or systemic steroids, aerosolized mucolytics, bronchoscopic lavage, direct bronchoscopic extraction and, in few reports, aerosolized fibrinolytic and pulmonary vasodilators. METHODS Four cases of PB after Fontan procedure occurred in our center from January 2008 to January 2012. RESULTS All patients showed preserved ventricle function and underwent embolization of systemic-pulmonary collaterals prior to a Fontan procedure and all had chylothorax in the perioperative period. Two patients died; 1 of massive thrombosis of the superior vena cava and right pulmonary artery and the other of acute asphyxiation and refractory cardiac failure. CONCLUSIONS The exact causes of PB in Fontan patients remain unknown. An early diagnosis and a multiple therapy with steroids, mucolytics, pulmonary vasodilators, and aerosolized tissue plasminogen activator may be effective.

Left ventricular assist device as destination therapy in cardiac end-stage dystrophinopathies: Midterm results

Objective: We report our experience with the use of a left ventricular assist device (LVAD) as destination therapy (DT) for the management of patients with cardiac end‐stage dystrophinopathies. Methods: From February 2011 to February 2016, 7 patients with dystrophinopathies and dilated cardiomyopathy (DCM) were treated with LVADs at our institution. Median age at surgery was 16.5 years (range, 14.2‐23.4 years). All patients were preoperatively evaluated by a multidisciplinary team approach. Results: All patients survived to hospital discharge. The early postoperative course was characterized by abdominal bleeding (1 patient) and retropharyngeal bleeding (1 patient). Because of abdominal or retropharyngeal bleeding, both required postoperative heparin infusion discontinuation for 35 and 33 days, respectively. Among the late complications, 1 child developed osteolysis and infection at the pedestal site of the device, which required surgical displacement; 1 patient required gastrostomy as a result of poor feeding, and another had a cerebral stroke, which was treated with percutaneous thrombus aspiration. The other 2 patients did not show early or late complications. At a median follow‐up time of 21.7 months (range, 3‐45 months) there have been 3 deaths: 1 patient died of a lung infection after 45 months, 1 died of tracheal bleeding after 29 months, and 1 died of cerebral hemorrhage after 14 months. Conclusions: Our experience indicates that the use of an LVAD as DT in patients with dystrophinopathies with end‐stage DCM is feasible, suggesting that it may be suitable as a palliative therapy for the treatment of these patients with no other therapeutic options.

Harmonic imaging with Levovist for transthoracic echocardiographic reconstruction of left ventricle in patients with post-ischemic left ventricular dysfunction and suboptimal acoustic windows

BACKGROUND Attempts to perform transthoracic 3-dimensional echocardiography (3DE) are often encumbered by poor definition of chamber borders in adult patients who have technically suboptimal acoustic windows. METHODS To assess whether harmonic imaging (HI) and contrast agents can facilitate transthoracic 3DE assessment of the left ventricle, we used fundamental imaging (FI), HI alone, and HI coupled with the echo-enhancing contrast agent Levovist in 15 consecutive patients with post-ischemic left ventricular (LV) dysfunction and technically difficult windows. Dynamic 3DE image data sets were obtained at 5-degree angles (36 slices) from a transthoracic apical view. From these data a total of 240 myocardial segments were analyzed with the use of dynamic short-axis paraplane slices at basal, middle, and apical LV levels (standard 16 segment model). For border definition, each segment was scored in random sequence on the following scale by 2 independent investigators: 0 = not seen, 1 = suboptimal visualization, and 2 = well defined. RESULTS Our results showed a significant increase in the number of well-visualized segments when harmonic mode combined with Levovist injection was compared with FI and HI alone. CONCLUSION Harmonic imaging alone improves LV assessment by 3DE when compared with FI. Contrast imaging in which Levovist is added to HI further improves the capability of transthoracic tomographic 3DE in the visualization of LV myocardial segments. This could allow 3DE by transthoracic windows to be used more widely in adults for the evaluation of LV volume and function.

A current approach to heart failure in Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is a genetic, progressive neuromuscular condition that is marked by the long-term muscle deterioration with significant implications of pulmonary and cardiac dysfunction. As such, end-stage heart failure (HF) in DMD is increasingly becoming the main cause of death in this population. The early detection of cardiomyopathy is often challenging, due to a long subclinical phase of ventricular dysfunction and difficulties in assessment of cardiovascular symptomatology in these patients who usually loose ambulation during the early adolescence. However, an early diagnosis of cardiovascular disease in patients with DMD is decisive since it allows a timely initiation of cardioprotective therapies that can mitigate HF symptoms and delay detrimental heart muscle remodelling. Echocardiography and ECG are standardly used for screening and detection of cardiovascular abnormalities in these patients, although these tools are not always adequate to detect an early, clinically asymptomatic phases of disease progression. In this regard, cardiovascular magnetic resonance (CMR) with late gadolinium enhancement is emerging as a promising method for the detection of early cardiac involvement in patients with DMD. The early detection of cardiac dysfunction allows the therapeutic institution of various classes of drugs such as corticosteroids, beta-blockers, ACE inhibitors, antimineralocorticoid diuretics and novel pharmacological and surgical solutions in the multimodal and multidisciplinary care for this group of patients. This review will focus on these challenges and available options for HF in patients with DMD.

Coronary plaque composition assessed by intravascular ultrasound virtual histology: association with long-term clinical outcomes after heart transplantation in young adult recipients.

To assess coronary plaque composition by virtual histology intravascular ultrasound (VH‐IVUS) analysis in young adult recipients and to correlate these findings with time from heart transplant (HTx) and long‐term outcomes.

Dystrophin Cardiomyopathies: Clinical Management, Molecular Pathogenesis and Evolution towards Precision Medicine

Duchenne’s muscular dystrophy is an X-linked neuromuscular disease that manifests as muscle atrophy and cardiomyopathy in young boys. However, a considerable percentage of carrier females are often diagnosed with cardiomyopathy at an advanced stage. Existing therapy is not disease-specific and has limited effect, thus many patients and symptomatic carrier females prematurely die due to heart failure. Early detection is one of the major challenges that muscular dystrophy patients, carrier females, family members and, research and medical teams face in the complex course of dystrophic cardiomyopathy management. Despite the widespread adoption of advanced imaging modalities such as cardiac magnetic resonance, there is much scope for refining the diagnosis and treatment of dystrophic cardiomyopathy. This comprehensive review will focus on the pertinent clinical aspects of cardiac disease in muscular dystrophy while also providing a detailed consideration of the known and developing concepts in the pathophysiology of muscular dystrophy and forthcoming therapeutic options.

Comment on: ‘Implantation of a left ventricular assist device to provide long term support for end-stage Duchenne muscular dystrophy-associated cardiomyopathy’ by Stoller et al.: Letter to the Editor

We read with great interest the case reported by Stoller and colleagues, regarding the implantation of a centrifugal continuous left ventricular assist device (LVAD) as a long-term support in a patient with Duchenne muscular dystrophy (DMD) presented with cardiogenic shock. The authors addressed an important issue: despite increased awareness among clinicians, heart failure (HF) is increasingly becoming the leading cause of death in DMD patients. Unfortunately, none of the available therapeutic options address the progressive deterioration of ventricular performance. Moreover, DMD has traditionally been considered as a relative contraindication for cardiac transplantation. A possible treatment for end-stage HF in these patients is the use of LVAD as a destination therapy (DT). Nevertheless, caution has to be exercised since the current International Society for Heart & Lung Transplantation (ISHLT) indication, conceived for chronic old patients, excludes patients with important disability. Therefore, the frail status in DMD patients is an important point to be addressed and discussed with every single patient, especially when defining the strategy in accordance with the family’s and caregivers’ expectations. Our group reported in 2012 the first two cases of successful LVAD implantation as DT in adolescents with DMD, using the Jarvik 2000 (Jarvik Heart, Inc, New York), a thumb-sized axial flow impeller pump. Since that time, we have treated seven DMD patients. All patients survived to hospital discharge: this represents the most extensive cohort of patients available. At a median follow-up time of 21.7 months (range, 3–45 months), there have been three deaths: one patient died of a lung infection after 45 months, one died of tracheal bleeding after 29 months, and one died of cerebral haemorrhage after 14 months. Remarkably, a patient survived at a follow-up of 1825 days: to the best of our knowledge, this is the longest follow-up available for a patient with an LVAD affected by DMD. Our current data are consistent with those presented by Stoller et al. and also by other groups, confirming the feasibility of using LVAD as DT in DMD subjects with HF. A multidisciplinary approach with careful evaluation of frailty and co-morbidities is crucial to assess the proper selection of DMD patients. Actually, a shared decision process is needed to obtain a collaborative contact with patient, parents, and caregivers, making this strategy successful. We strongly believe that DMD patients deserve LVAD as DT, and so we encourage other groups to consider this therapeutic strategy and share it with patients, family, and all caregivers as a part of palliative strategy.

The Use of Berlin Heart EXCOR VAD in Children Less than 10 kg: A Single Center Experience

Objective: Despite the improvement in ventricular assist device (VAD) therapy in adults and in adolescents, in infant population only Berlin Heart EXCOR (BHE) is licensed as long term VAD to bridge children to Heart Transplantation (HTx). Particularly demanding in terms of morbidity and mortality are smallest patients namely the ones implanted in the first year of life or with a lower body surface area. This work aims at retrospective reviewing a single center experience in using BHE in children with a body weight under 10 kg. Methods: Data of all pediatric patients under 10 kg undergoing BHE implantation in our institution from March 2002 to March 2016 were retrospectively reviewed. Results: Of the 30 patients enrolled in the study, 53% were male, 87% were affected by a dilated cardiomyopathy with an average weight and age at the implantation of 6.75 ± 2.16 Kg and 11.57 ± 10.12 months, respectively. Three patients (10%) required a BIVAD implantation. After the implantation, 7 patients (23%) required re-intervention for bleeding and 9 patients (30%) experienced BHE cannulas infection. A total of 56 BHE pump were changed for thrombus formation (1.86 BHE pump for patient). The average duration of VAD support was 132.8 ± 94.4 days. Twenty patients (67%) were successfully transplanted and 10 patients (33%) died: 7 for major neurological complication and 3 for sepsis. Conclusion: Mechanical support in smaller children with end stage heart failure is an effective strategy for bridging patients to HTx. The need for BIVAD was relegated, in the last years, only to restrictive cardiomiopathy. Further efforts are required in small infants to improve anticoagulation strategy to reduce neurological events and BHE pump changes.