Pietro Angelo Abbruzzese

Aortic coarctation: an overview

In severe aortic coarctation in the neonatal period, surgical repair is required soon after clinical stabilization. Elective repair of isolated aortic coarctation is nowadays indicated at 3-6 months of life or at the time of diagnosis. At present, no single operation appears to have a clear superiority. However, during the first months of life, an extended end-to-end anastomosis is considered the best option by most authors, even though weight at operation and anatomy of the aortic arch are also significant determinants of late recoarctation. In cases of aortic arch hypoplasia, which occurs in up to 70% of neonatal and infant coarctations, especially when associated anomalies are present, surgery seems the treatment of choice. After 3 months of age and in the adult population, balloon angioplasty and stent placement are considered a suitable option. Recently, we adopted a median sternotomy approach without the use of extracorporeal circulation for the treatment of aortic coarctation with a hypoplastic aortic arch. We treated 11 patients with satisfactory results at an average follow-up of 40 months.

Regurgitant Flow of Mitral Valve Prostheses: An Intraoperative Transesophageal Echocardiographic Study

To assess the regurgitant characteristics of mitral biologic and mechanical prostheses immediately after implantation, intraoperative transesophageal echocardiography was performed in 27 patients, aged 32 to 69 years, undergoing open-heart surgery for rheumatic heart disease (n = 19), mitral valve prolapse (n = 3), malfunctioning prostheses (n = 3), or periprosthetic leaks (n = 2). The prostheses included 13 biologic (Carpentier-Edwards) and 14 mechanical valves (five Starr-Edwards, five Medtronic-Hall, and four Bjork-Shiley). Physiologic transvalvular regurgitant flow was detected in both biologic and mechanical prostheses. The spatial extent of the regurgitant jets was usually greater in the mechanical than in the biologic valves, and systolic jets, characteristic of each type of valve, were visualized consistently. Trivial periprosthetic jets (PPJs) were observed in many implanted valves (14/27). The median maximal jet area was 0.46 cm2 (range 0.1 to 1.5 cm2). Cardiopulmonary bypass was reinstituted in two patients. In one patient a PPJ was judged extensive enough (area 3.6 cm2) to warrant surgical revision of the implant, but no dehiscence was found. In the other patient a turbulent PPJ (area 5.5 cm2) was associated with a 0.5 cm dehiscence at the surgical inspection. In conclusion, (1) all mitral prostheses exhibit physiologic transvalvular regurgitation, (2) trivial mitral PPJ is a common finding in newly implanted mitral valves and does not require the revision of the implant, and (3) further experience based on larger series of patients is required to determine the maximal acceptable size of a mitral PPJ detected by intraoperative transesophageal echocardiography.

Asymptomatic inflammatory myofibroblastic tumor of the heart: immunohistochemical profile, differential diagnosis, and review of the literature

BACKGROUND Inflammatory myofibroblastic tumor (IMT) is an uncommon lesion, mainly occurring in children and young adults and extremely rare in the heart. IMTs are composed of differentiated myofibroblastic cells accompanied by inflammatory cells. Cardiac IMTs are considered biologically benign, but they may have fatal consequences depending upon the peculiarity of site. Because of their rarity in the heart, most knowledge is based on extracardiac lesions that have uncertain behaviour. METHODS AND RESULTS We investigated the morphologic features and the immunohistochemical profile of an intracardiac IMT, arising in the right outflow tract of an asymptomatic 11-month-old boy, by using a large panel of antibodies, many of them previously reported in extracardiac IMTs only. Results were compared with data of literature. After complete surgical excision of the tumor, the patient is disease-free at 1 year of follow-up. CONCLUSIONS The present case showed morphologic and immunohistochemical features characteristic of IMT. Immunohistochemistry was helpful for characterization and differential diagnosis. The immunoreactivity pattern (including calponin expression) was similar to that of extracardiac IMTs except for anaplastic lymphoma kinase 1 immunoreactivity, lacking in this benign intracardiac IMT but usually associated to favourable prognosis in extracardiac IMTs.

Detection of microbubbles released by oxygenators during cardiopulmonary bypass by intraoperative transesophageal echocardiography

Abstract Despite the improvements in cardiopulmonary bypass techniques, release of microbubbles in the systemic arterial circulation still occurs. It is believed that microemboli, prolonged arterial hypotension, defective cerebral blood flow autoregulation and nonpulsatile flow during cardiopulmonary bypass play a role in determining neurologic damage after cardiopulmonary bypass.1,2 Gaseous and particulate microemboli may originate from the pump-oxygenator system as well as from the cardiac chambers and pulmonary veins.1,3 In this study, transesophageal echocardiography was used to detect microbubbles reaching the arterial circulation during cardiopulmonary bypass. Two different types of oxygenators (bubbles and hollow fibers) were used to assess differences in their production of microbubbles.

Echocardiographic assessment of aortic valve replacement with stentless porcine xenografts

Stentless porcine xenografts (SPXs) implanted in the aortic position have potential hemodynamic advantages over traditional valve prostheses because of the lack of a rigid stent. Twenty-four patients (mean age 59 years) who underwent aortic valve replacement with SPXs were studied by echocardiography early after and 26 +/- 10 months (range 8 to 40) after operation. Peak and mean gradients, as well as aortic valve area, did not change significantly from baseline (16.3 +/- 8 and 9.8 +/- 5.6 mm Hg, and 1.78 +/- 0.63 cm2, respectively) to follow-up study (12.5 +/- 5 and 7.7 +/- 3 mm Hg, and 1.8 +/- 0.65 cm2, respectively). At baseline, color flow Doppler imaging showed aortic valve regurgitation where the leaflets coapted centrally in 17 of 24 patients (trivial, n = 14; mild, n = 3). Besides the central leak, paravalvular regurgitation was seen in 4 patients (trivial, n = 3; mild, n = 1). At follow-up, 18 of 24 patients had aortic valve regurgitation (trivial, n = 11; mild, n = 6; and moderate, n = 1). New valvular regurgitation (graded as trivial, n = 2; mild, n = 2; and moderate, n = 1) was detected in 5 patients, and new paravalvular regurgitation (graded as mild) developed in 1 patient. Two patients underwent repeat operation for valve-related complications: (1) rupture of a valve cusp with acute pulmonary edema, and (2) fibrotic stenosis of the left coronary ostium with unstable angina. In conclusion, this study demonstrates good hemodynamic performance of the SPX in the aortic position.(ABSTRACT TRUNCATED AT 250 WORDS)

Life-Threatening Tumors of the Heart in Fetal and Postnatal Age

OBJECTIVES To evaluate the role of histology in diagnosis and management of biologically benign heart tumors causing life-threatening symptoms and even death in children and fetuses. The clinical impact of a multidisciplinary approach including 2-D echocardiography, histology, genetics, and cardiac surgery has not yet been fully elucidated. STUDY DESIGN Forty-one consecutive antenatal (n = 17) or postnatal (n = 24) detected cardiac masses were evaluated by 2-D echocardiography (in alive patients) or at autopsy, and 12/41 cases with definite histologic diagnosis of primary and benign cardiac tumor were entered in this study. RESULTS Rhabdomyomas (n = 6), hemangiomas (n = 3), central fibrous body chondroma (n = 1), fibroma (n = 1), or left atrial myxoma (n = 1) were histologically diagnosed in 4 fetuses and in 8 children. Death occurred in 6 patients showing diffuse or infiltrative tumors, 2/6 experiencing intrauterine death or sudden and unexpected infant death. Seven patients underwent surgery, 4/7 are alive and well at >5 years follow-up, whereas 3 deaths followed partial tumor resection. Two fetuses with extensive tumor/s were aborted. Tuberous sclerosis complex gene mutations were seen in patients with rhabdomyomas. CONCLUSIONS Histology represents the best diagnostic approach in life-threatening pediatric cardiac tumors allowing definite diagnosis in cases other than rhabdomyoma and in sudden deaths, influencing clinical management and counselling. 2-D echocardiography remains the main tool for early clinical diagnosis and follow-up. A multidisciplinary approach is advisable because of rarity, difficult management, and possible associations with inheritable diseases.

Berry syndrome, a complex aortopulmonary malformation : One-stage repair in a neonate

Successful one-stage repair of a Berry syndrome (interrupted aortic arch, distal aortopulmonary septal defect, right pulmonary artery branch originating from the ascending aorta, and intact ventricular septum) in the neonatal period has been reported in only 2 cases. We report the case of a newborn operated on with deep hypothermic arrest and isolated myocardial perfusion in whom the interrupted aortic arch was corrected by direct anastomosis between the ascending and descending aorta and the aortopulmonary septal defect was treated with reconstruction of the pulmonary trunk and right pulmonary artery, using a flap of aortic tissue. A native pericardial patch was used to reconstruct the ascending aorta.

Pulmonary Valve Replacement With Mechanical Prosthesis: Long-Term Results in 4 Patients

Pulmonary valve replacement is rarely performed and is usually done with biological prostheses or grafts. The use of mechanical prostheses is still a debated issue. We analyzed the outcome of 4 patients at 11 years after pulmonary valve replacement with a mechanical prosthesis. During follow-up, all patients remained asymptomatic, with no arrhythmias and good anticoagulation. An echocardiogram showed preserved ventricular contraction and normal function of the prostheses, with low pressure gradients. No infective endocarditis occurred. Mechanical prostheses appear to be a good choice for pulmonary valve replacement. Surgical and clinical results were satisfactory with no complications, acceptable pressure gradients, and good ventricular function after more than 10 years. Lifelong anticoagulation is mandatory, but this is usually well tolerated.

Repair of an Unusual Aortic Coarctation Using an Extracellular Matrix Patch

The surgical treatment of neonatal aortic coarctation is usually accomplished with a termino-terminal anastomosis or a subclavian flap. The use of a patch to enlarge the isthmal narrowing may be an alternative but is frequently complicated by aneurysmal dilatation on the aortic wall opposite to the patch, probably because it disrupts the vascular anatomic integrity. Extracellular matrix patches promise to restore the original tissue structure and could therefore be a valid alternative to other materials. We describe an aortic coarctation with an uncommon anatomic aspect treated with a CorMatrix (CorMatrix, Alpharetta, GA) extracellular matrix patch.

Use of Oversized Injectable Valves in Growing Children for Total Repair of Right Ventricular Outflow Tract Anomalies (Preliminary Results)

Right ventricular outflow tract surgery was originally confined to transannular patching, in the belief that pulmonary regurgitation was well tolerated. Because follow-up evaluations revealed the deleterious effects of pulmonary regurgitation, surgery today aims to spare or replace the valve. Available replacement devices have short lifetimes, considering growth mismatch in children. We hypothesize that oversizing the right infundibulum anticipates growth and that a squeezed prosthesis can complete the expansion process. The No-React® Injectable BioPulmonic Valve is designed for right infundibular surgery in adults, and hundreds of implants have shown promising results. We used this device for surgery in babies, with the addition of an innovative oversizing technique. This study evaluates our preliminary results and investigates whether such a technique might reduce growth mismatch. From September 2010 through July 2012, we implanted 11 injectable pulmonic valves. The median age of our patients was 23 months. After opening the right infundibulum, we enlarged it as much as possible with a wide patch. Before completing the patch suture, we injected an oversized valve. No problems occurred during surgery. No major insufficiency or leak was observed. We conclude that prostheses can be quite oversized and perform well even when not completely expanded. Oversized injectable pulmonic valves, shrunken to a smaller diameter, enabled the implantation of a device wider than otherwise possible, without affecting performance. Moreover, the prosthesis tended to return to its original size following growth, thereby reducing growth mismatch. Longer follow-up and larger numbers of patients are needed for verification.