Angelo Micheletti

A comparison between the early and mid-term results of surgical as opposed to percutaneous closure of defects in the oval fossa in children aged less than 6 years

OBJECTIVES To compare surgical as opposed to percutaneous interventional closure of isolated atrial septal defects in the oval fossa in terms of hospital stay, efficacy, and complications, and to study the respective role of the two techniques in current practice. METHODS Between January 1998 and April 2004, 126 out of 1210 patients treated at our institution for closure of an isolated defect in the oval fossa were aged less than 6 years. The mean age of these 126 patients at procedure was 4.2 plus or minus 1 year. The ratio of females to males was 74 to 52. RESULTS Of the patients, 62% were treated successfully using a percutaneous approach. The groups treated surgically or percutaneously did not differ for age, gender, or indications for treatment. No deaths occurred. The rates of total and major complications were higher in the group undergoing surgical closure, at 34% versus 9%, p less than 0.0001, and 10.5% versus 1%, p equal to 0.01, respectively. Embolisation of the device requiring subsequent surgery occurred in 1% of patients. The stay in hospital was shorter in those closed percutaneously, at 3.2 plus or minus 0.9 days versus 6.8 plus or minus 2.8 days, p equal to 0.0001. During a mean follow-up of 3.4 plus or minus 1.9 years, no major complications occurred in either group, and symptoms improved significantly in both groups. Additional sequels occurred in 2 patients who had major complications subsequent to surgical closure. CONCLUSIONS Even in young children, it is both feasible and safe to close defects in the oval fossa percutaneously. Compared to surgical closure, the transcatheter approach allows a shorter stay in hospital, and has a lower rate of complications. Early and mid-term follow-up has confirmed the safety and efficacy of both techniques.

Right and Left Ventricular Strain and Strain Rate in Young Adults before and after Percutaneous Atrial Septal Defect Closure

To evaluate acute change of right and left ventricle after percutaneous closure of isolated atrial septal defect (ASD) 21 adult patients (13 F; 8 M) aged 28 ± 9.5 range 18–49 years have been examined by echocardiography before and 24 hours after percutaneous closure of ASD. Twenty‐one normal adult subjects, as control group were included. A MyLab25 echo machine equipped with a multifrequency 2.5–3.5 MHz transducer was used. Offline computer‐based analysis for strain and SR were performed using XStrain software based on a feature tracking algorithm. All patients had ASD OS2 with right ventricular dilatation and diastolic areas were larger than in controls: P = 0.0158. Global right ventricular longitudinal strain was higher P = 0.0438. Twenty‐four hours after ASD closure, right ventricular diastolic and systolic areas were significantly reduced. Right ventricular global longitudinal systolic strain decreased: P = 0.00016, as well as global right ventricular longitudinal SR –1.56/sec ± 0.57 vs. –1.28/sec ± 0.31, P = 0.02646. At the mean time left ventricular end diastolic volume and left ventricular cardiac output measured by two‐dimensional echocardiography both increased significantly P = 0.002145 and 0.013409. Global circumferential strain at mitral level augmented significantly –20.3%± 4.64 vs. –25.39%± 5.22, P = 0.00003. Longitudinal strain of the right ventricle works as indicator of right ventricular function dependent on loading conditions while SR seems to be less dependent on it. Circumferential strain could be used as an indicator of left ventricular response to normalized loading conditions. (Echocardiography 2011;28:730‐737)

Timing of pulmonary valve replacement after tetralogy of Fallot repair.

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. If left untreated, it carries a 33% mortality in the first year of life and a 50% mortality in the first 3 years of life. Since the introduction of the first open-heart repair by Lillehei and Varco in 1954, surgical management of TOF has evolved to be the primary repair during infancy in the majority of patients. Surgical management of TOF results in anatomic and functional abnormalities in the majority of patients, such as chronic pulmonary valve regurgitation and right ventricular (RV) dysfunction. Long-standing chronic pulmonary valve regurgitation can result in RV dilatation and failure, increasing tricuspid regurgitation, impaired exercise performance and supraventricular or ventricular arrhythmias. A timely reoperation may prevent these consequences, with a complete RV-function recovery. This article provides insight into the questions of when to perform a pulmonary valve implantation and in whom.

Covered-stent implantation to treat aortic coarctation

Aortic coarctation comprises approximately 7% of all known congenital heart defects. Surgery and balloon angioplasty have been performed for many years but are associated with a significant incidence of recoarctation and aneurysm formation. Although bare-stent implantation decreases the incidence of recoarctation, the risk of aortic dissection or aneurysm formation is not eliminated. Described initially to treat patients with coexistent aneurysm of the aortic wall, we currently believe that covered-stent implantation for aortic coarctation should play a more important role. Our increasing experience from expanding their indication to deal with complications from previous interventions, associated defects or particular anatomical situations has led us to conclude that most of cases of aortic coarctation in adolescents and adults should be treated by the implantation of covered stents.

The prenatal diagnosis of, and short-term outcome for, patients with congenitally corrected transposition.

Congenitally corrected transposition is a rare congenital anomaly, with only a few cases diagnosed and reported prenatally even in the largest fetal series. To determine the morphologic features and outcome for the lesion as recognized during fetal life, we reviewed the fetal and postnatal echocardiograms and medical records of 11 consecutive cases of congenitally corrected transposition. These were identified among 230 (4.7%) consecutive cases of structural cardiac disease referred to our fetal cardiology unit over a period of 4 years. The mean gestational age at diagnosis was 24.7 weeks. Reasons for referral were suspected complete transposition, abnormal position of the heart, and bradyarrhythmias. Associated cardiac lesions included an abnormal cardiac position in 6 cases, ventricular septal defect in 8, obstruction of the subpulmonary outflow tract in 6, tricuspid valvar displacement in 5, and complete atrioventricular block in 2. Only 3 of the cases had mild tricuspid regurgitation prior to birth. Termination was chosen in 4 cases with severe obstruction to pulmonary flow. Of the remaining cases, 2 patients died at 3 and 12 months after birth, respectively. Both developed significant tricuspid regurgitation associated with unexpected major arrhythmias. The remaining 5 patients are alive and relatively well at a mean follow-up of 25.4 months. An epicardial pacemaker was inserted in 1 because of complete atrioventricular block. We conclude that prenatal counseling must be guarded following the diagnosis of congenitally corrected transposition, even in fetuses with an apparently favorable state at initial examination. Some of these cases may undergo major and unexpected changes, particularly with regard to cardiac rhythm and tricuspid valvar function, with concomitant significant changes in prognosis.

Echocardiographic assessment after surgical repair of tetralogy of fallot.

Surgical correction of tetralogy of Fallot is still one of the most frequently performed intervention in pediatric cardiac surgery, and in many cases, it is far from being a complete and definitive correction. It is rather an excellent palliation that solves the problem of cyanosis, but predisposes the patients to medical and surgical complications during follow-up. The decision-making process regarding the treatment of late sequel is among the most discussed topics in adult congenital cardiology. In post-operative Fallot patients, echocardiography is used as the first method of diagnostic imaging and currently allows both a qualitative observation of the anatomical alterations and a detailed quantification of right ventricular volumes and function, of the right ventricular outflow tract, and of the pulmonary valve and pulmonary arteries. The literature introduced many quantitative echocardiographic criteria useful for the understanding of the pathophysiological mechanisms involving the right ventricle and those have made much more objective any decision-making processes.

Role of imaging in interventions on structural heart disease

Recent technological progresses have led to the development of new devices and procedures which have greatly improved the chance to effectively treat structural heart diseases in both children and adults. Interventional cardiology has been receiving fast and wide implementation as an effective alternative treatment to surgery for several congenital and acquired diseases. The advent of transcatheter valve implantation/repair techniques constitutes one of the main breakthroughs of the last decades. Such development and implementation is strictly related to a continuous progress in cardiac imaging as well. Indeed, multimodality cardiac imaging (such as X-ray, echocardiography, MRI, multidetector computed tomography) has become essential in providing accurate patient selection and in monitoring the interventional procedures in order to optimize the success rate and minimize the frequency of complications. The current article aims at reviewing the role of multimodality imaging for planning and guiding interventions in several structural heart diseases.

Percutaneous pulmonary valve implantation in a single artery branch: A preliminary experience.

To describe preliminary experience of percutaneous pulmonary valve implantation, in a single pulmonary branch position. Two procedures in 2 patients from a single center are described, where implantation of percutaneous valves within a single pulmonary artery branch was technically successful. The procedural indication was pulmonary valve regurgitation and/or residual stenosis. The 2 patients were symptomatic. An Edwards Sapien™ valve (Patient 1), and a Medtronic Melody™ valve (Patient 2) were implanted. Both pts were discharged with an excellent valve function. In this report it is underlined that this modality is technically feasible and may be considered an option in patients with congenital heart defect under special circumstances.

Treatment of right ventricular outflow tract dysfunction: a multimodality approach

The right timing to replace the pulmonary valve in a patient with dysfunction of the right ventricular outflow tract is unknown. Both percutaneous pulmonary valve and surgical prosthesis are suitable options. In every patient, the right ventricle (RV) remodels and recovers differently after pulmonary replacement. Therefore, it is difficult to identify the best treatment option and to predict the long-term results. In the last few years, we focused our research on optimizing the characterization of these patients through advanced cardiovascular imaging in order to find possible variables, parameters, and reproducible measurements that can help us in the decision-making process. The aim of the present article is to present our ongoing research lines that focus on the characterization and optimal treatment approach to the dysfunction of the RVOT.

The Challenging Pathway Toward Heart Transplant Listing for Adult Congenital Heart Disease Patients: HEART TRANSPLANT LISTING FOR ACHD PATIENTS

Adult congenital heart disease (ACHD) patients are at risk for end-stage heart failure; heart transplantation (Htx) represents the only definitive therapy available although not easily achievable for all patients. The study aims to assess the pathway difficulties and outcomes of ACHD patients with end-stage heart failure referred for Htx evaluation. This is a single center retrospective study on ACHD patients with end-stage heart failure referred to Htx evaluation from 2004 to 2015. Demographic data, medical history, failure modality, and follow-up were obtained from patient charts. End-points were Htx list enrollment, transplant, and survival. Statistical analysis was performed comparing patients listed and not listed. There were 21 ACHD patients with end-stage heart failure referred to Htx evaluation. Transplant listing was declined for 12 (57%) meanwhile 9 patients were listed. Htx was successfully achieved in 3 patients after 24 and 36 months, respectively. Three patients are still on the wait list and three died while waiting, with a listed group mortality of 33.3% (3/9). Mortality occurred in first 18 months after Htx list enrollment. Not listed group mortality was 50% (6/12) and occurred after a median time of 17.5 months (IQR: 9-23 months). There was no difference in survival (P = 0.574) between listed and not listed (89, 63, and 63% vs. 83, 56, and 47% at 12-24-48 months). Follow-up median duration was 27 months (IQR: 14-56 months). Heart transplant listing for ACHD patients with end-stage heart failure is hard to obtain. Almost 2/3 of the patients were declined. Survival for these patients is reduced severely either in waiting list for transplant or excluded indicating the potential need of mechanical circulatory support as bridge to transplant or as destination therapy to improve survival likelihood.