Is the short axis view of the fetal heart useful in improving the diagnostic accuracy of outlet ventricular septal defects?

OBJECTIVES: Outlet ventricular septal defects (VSDs) are usually suspected on the five-chamber view of the fetal heart; however, postnatal confirmation occurs only in a small number of cases. The aim of this study was to evaluate if the systematic assessment of the short axis view may improve the prediction of prenatally detected outlet VSDs. METHODS: Cases where isolated outlet VSD was suspected on the five-chamber view were assessed by using the short axis scanning plane for confirmation of the defect. Postnatal assessment was performed within 2 weeks from birth at our Paediatric Cardiology Unit. RESULTS: An outlet VSD was suspected at five-chamber view in 23 fetuses. Postnatal confirmation of the VSD occurred in 14 cases where the defect was prenatally detected both on the five chamber and the short axis views. VSDs were not confirmed at postnatal assessment only in two cases where outlet VSD was suspected on both views, whereas all the seven cases with a suspected VSD on the five-chamber view only turned out to be false positives. CONCLUSION: The short axis view of the fetal heart seems useful in confirming the presence of outlet VSDs. © 2016 John Wiley & Sons, Ltd.

Functional tricuspid valve regurgitation in adults with congenital heart disease: an emerging problem.

BACKGROUND AND AIM OF THE STUDY: At present, limited data are available regarding functional tricuspid regurgitation (TR) in adults with congenital heart disease (ACHD). Functional TR is the consequence of right ventricular (RV) dilation or dysfunction that, in these patients, is typically associated with chronic RV volume overloading. The study aim was to resolve this problem by evaluating results obtained from patients after surgical treatment of ACHD. METHODS: A total of 65 patients (mean age 46 years) with ACHD underwent an operation to correct functional TR during elective cardiac surgery between January 2000 and December 2008 at the authors' institution. The preoperative median NYHA functional class was 3, and the median TR grade was 3. Surgical treatment of the primary cardiac lesion included atrial septal defect (ASD) closure in 40 patients and pulmonary valve implantation in 25. Functional TR was treated by annuloplasty (n = 48), rigid tricuspid valve ring (n = 14), or tricuspid valve replacement (n = 3). RESULTS: There were no in-hospital deaths. The median TR grade at discharge was 0. During a mean follow up period of 63 months (range: 12-96 months) there was one delayed death that was not cardiac-related. Both, the NYHA functional class (p = 0.001) and TR grade (p = 0.001) were significantly improved among survivors. One patient (1.5%) had tricuspid valve replacement at five years after annuloplasty. No significant differences regarding annuloplasty versus rigid tricuspid valve ring were noted during the follow up period. CONCLUSION: Functional TR is an emerging, though as yet still underestimated, problem in ACHD. It is a consequence of RV dilation/dysfunction that, in these patients, is typically related to chronic RV volume overloading. Surgery should be considered to resolve this problem, and can be performed at low risk and with good mid-term results.

Infective endocarditis following body piercing: Presentation of one case due to Gemella morbillorum and review of the literature.

BACKGROUND: In the last decade, body piercing has gained strongly in popularity. There are a few reports in the literature regarding infective endocarditis after body piercing. CASE REPORT: The case of an 18-year-old girl with heart failure and a six-month history of recurring fever is presented. Clinical and laboratory evaluation led to the diagnose of infective endocarditis due to Gemella morbillorum. Oral piercing was recognized to be the cause of bacteremia. Fourteen cases of infective endocarditis related to body piercing were found in the literature and the clinical, etiologic, pathogenic, and preventive aspects of this topic are presented. CONCLUSIONS: Infective endocarditis may be a complication of body piercing and has been observed in patients with heart lesions as well as in previous healthy people. Infective endocarditis can follow body piercing involving either mucous membranes or the skin. This patient is the first case of Gemella morbillorum endocarditis after body piercing reported in the literature. Careful observation for infection at the site of piercing and its treatment as well as professional training for operators seem to be the most effective way to minimize the risk of infective endocarditis after body piercing.

Syncope in children and adolescents: a two-year experience at the Department of Paediatrics in Parma.

BACKGROUND AND AIM OF THE WORK: Syncope occurs frequently in childhood and adolescence. It generally is benign, but may be a sign of pathology. The purpose of this study is to determine the incidence of syncope in children and adolescents in the Parma area, analyse the frequency of syncope due to cardiac, neurological or metabolic diseases, to identify a diagnostic procedure based on careful review, clinical features, and results of investigations. METHODS: The records of 156 children (age 1 to 18 years, mean age 10.5), who were referred to the Paediatric Department for syncope were reviewed. RESULTS: The incidence of syncope in the paediatric population of Parma during a 2-year period (2005-2006) resulted in 86.5/100.000 per year. The cause of syncope was discovered in 154/156 cases. Cardiovascular syncope was diagnosed in 121/156 cases (77.5%). Neurological syncope was diagnosed in 31/156 cases (20%), while metabolic syncope was diagnosed in 2/156 cases (1.25%). The most frequent type was the (benign) neurocardiogenic syncope, but 3 cases were due to arrhythmia, 9 cases were due to epilepsy, 1 case was due to subarachnoid hemorrage and 2 cases were due to hypoglicemia. CONCLUSIONS: Syncope frequently requires medical examination or admission to paediatric departments and, in the majority of cases, does not subtend serious diseases. Nevertheless, syncope always requires a complete medical evaluation in order to exclude underlying pathology. An electrocardiogram must complete the initial evaluation of all patients with syncope. Other investigations are required if clinical data and the ECG do not provide an etiological explanation of the episode.

How often an isolated cardiac disproportion predicts a coarctation of the aorta? Single center experience and systematic review of the literature.

BACKGROUND: Cardiac disproportion is considered as an indirect sign of coarctation of the aorta (CoA). In this review, we have reassessed the positive predictive value (PPV) of such finding for a postnatal confirmation of CoA. DATA SOURCES: All cases of isolated cardiac disproportion diagnosed in the four-chamber and/or three-vessel/three-vessel and trachea views (right/left sections >1.5) were included. Postnatal cardiac findings were recorded. Additionally, a systematic literature search (PubMed, EMBASE, Cochrane library, and the reference lists of identified articles) regarding the association between antenatally detected cardiac disproportion and postnatal confirmation of CoA was performed. Data from our center were pooled with those derived from the literature. RESULTS: Ten fetuses with isolated cardiac disproportion were selected from our center and 259 from the literature review. CoA was postnatally confirmed in 101/269 (PPV 38%). PPV of antenatal cardiac findings was significantly higher in earlier gestation (23/27, 85% <26+0 weeks versus 11/39, 28%≥26+0 weeks, p < .001). No significant difference was noticed comparing ventricular disproportion with combined ventricular and great vessels disproportion (86/230 versus 15/39, p .89). DISCUSSION: Isolated cardiac disproportion has an overall chance of one in three of heralding a CoA in the neonate. The specificity of these findings is significantly higher in the second trimester.

Paradoxical emboli in children and young adults: role of atrial septal defect and patent foramen ovale device closure.

OBJECTIVE: To describe a multicenter experience with patent foramen ovale (PFO) and atrial septal defect (ASD) device closure for presumed paradoxical emboli in children and young adults (<35 years old). PATIENTS AND METHODS: Medical records were reviewed of patients who had device closure of an ASD or PFO, who were younger than 35 years, and who had a history of presumed paradoxical embolus between January 1999 and August 2005 at Mayo Clinic, Rochester, Minn, University of Parma, Parma, Italy, and Loyola University Medical Center, Maywood, Ill. RESULTS: Forty-five patients fulfilled the inclusion criteria. Median patient age was 29.0 years (range, 5.0-34.9 years), and 23 patients (51%) were male. Clinical diagnoses included the following: stroke, 30 (67%); transient ischemic attack, 13 (29%); myocardial infarction, 1 (2%); and renal infarct, 1 (2%). Overall, 42 patients (93%) had a PFO, and 3 (7%) had an ASD. Seventeen patients had known cardiovascular disease risk factors: tobacco use (10 patients), hypercoagulable states (7 patients), systemic hypertension (3 patients), and hyperilpidemia (2 patients). No major procedural complications occurred. Median follow-up evaluation was performed at 5.3 months (range, 2.5-40.0 months). Forty-four patients (98%) had no recurrent neurologic events and no residual atrial shunt by contrast transthoracic echocardiography. CONCLUSIONS: Cryptogenic ischemic events occur in young patients and have serious sequelae. The potential for paradoxical embolization through a PFO or an ASD should be assessed in all such patients. In our short-term follow-up, device closure was a safe alternative therapeutic option for children and young adults with presumed paradoxical emboli.

[Transcatheter treatment of patent ductus arteriosus]. / Trattamento transcatetere del dotto arterioso pervio.

Isolated patent ductus arteriosus accounts for 10 to 18% of all cardiovascular malformations. Transcatheter closure of the patent ductus arteriosus has now become established practice with a high success rate and a low incidence of complications. Many devices have been proposed during the last 20 years. The use of some of these devices has largely been abandoned for different reasons: high costs, the need of large transvenous sheaths and the high incidence of late residual shunts. The devices currently available (Amplatzer duct occluder, buttoned device, DuctOcclud coil, Gianturco-Grifka vascular occlusion device, detachable coils) render the embolization of a patent ductus arteriosus relatively easy, with a short hospital stay. We feel that this is the treatment of choice for the majority of patients with a patent ductus arteriosus. The aim of this article was to review our experience and report the state-of-the-art in transcatheter patent ductus arteriosus therapy.

Pulmonary arterial hypertension in childhood: an unsual presentation with fibrosing mediastinitis.

Acquired stenosis of normally connected pulmonary veins is a rare condition in children, usually associated with mediastinal processes. It may present later with a less specific clinical picture, symptoms and signs mimicking chronic lung disease. Fibrosing mediastinitis is a rarer disorder of unknown etiology, although several suspected causes such as granulomatous diseases, characterized by fibrous tissue proliferation within the mediastinum, leading to respiratory and cardiac failure by bronchial obstruction or pulmonary hypertension.

Glycopyrrolate and theophylline for the treatment of severe pallid breath-holding spells.

Severe pallid breath-holding spells (BHSs) are based on parasympathetic hyperactivity, leading to cardiac asystole, pallor, brain ischemia, loss of consciousness, and reflex anoxic seizures. In recent years, an increasing number of patients with severe pallid BHSs have been successfully treated with pacemaker implantation. We present the case of a 13-month-old girl suffering from repeated severe pallid BHSs, causing asystole, loss of consciousness, and generalized anoxic seizures. She underwent treatment with oral glycopyrrolate, an anticholinergic drug, and an oral retard preparation of theophylline. The aim of the treatment was to decrease cardiac inhibition with glycopyrrolate and to bring about a positive chronotropic effect with theophylline. In our case, the combined therapy was effective in suppressing syncope and reflex anoxic seizures associated with BHSs This avoided the need for ventricular pacemaker implantation.

Adams-Stokes attack as the first symptom of acute rheumatic fever: report of an adolescent case and review of the literature.

BACKGROUND: Acquired complete heart block, in pediatric age is mainly the results of direct injury to conduction tissue during cardiac surgery or cardiac catheterisation. It can also be observed in different clinical settings as infectious diseases, neoplasia, and inflammatory diseases. It has a wide range of presentation and in some settings it can appear a dramatic event. Although a rare finding during acute rheumatic fever, with a transient course, it may need a specific and intensive treatment. CASE PRESENTATION: We report the case of an Adams-Stokes attack in an adolescent with acute rheumatic carditis and complete atrio-ventricular block. The attack was the first symptom of carditis.We reviewed the literature and could find 25 cases of complete atrio-ventricular block due to rheumatic fever. Ten of the 25 patients experienced an Adams-Stokes attack. Nineteen of the 25 patients were certainly in the pediatric age group. Seven of the 19 pediatric cases experienced an Adams-Stokes attack. In 16/25 cases, the duration of the atrio-ventricular block was reported: it lasted from a few minutes to ten days. Pacemaker implantation was necessary in 7 cases. CONCLUSION: Rheumatic fever must be kept in mind in the diagnostic work-up of patients with acquired complete atrio-ventricular block, particularly when it occurs in pediatric patients. The insertion of a temporary pacemaker should be considered when complete atrio-ventricular block determines Adams-Stokes attacks. Complete heart block during acute rheumatic fever is rare and is usually transient. Along with endocarditis, myocarditis and pericarditis, complete atrio-ventricular block has been recognized, rarely, during the course of acute rheumatic carditis.

Atipical Kawasaki disease with coronary aneurysm in infant.

Kawasaki disease is an acute febrile disease of unknown etiology, characterized by systemic vascular inflammation involving the small and medium sized arteries, with a predilection for the coronary arteries. It represents the leading cause of acquired heart diseases in children in developed countries. Diagnosis, difficult because of the clinical characteristics of the disease with typical signs and symptoms appearing sequentially and not simultaneously, may be even more complicated in case of unusual presentation, leading to delay in recognition, particularly in infant in whom a higher incidence of coronary arteries aneurysms has been reported. A high index of suspicion of Kawasaki disease must be maintained in case of prolonged fever in these patients. Timely appropriate treatment is essential to avoid severe sequels. We report the case of a 2 months old male infant with persistent febrile episode, transferred to us from another institution, who presented on echocardiography giant aneurysms on both coronary arteries.

Growth factor-induced mobilization of cardiac progenitor cells reduces the risk of arrhythmias, in a rat model of chronic myocardial infarction.

Heart repair by stem cell treatment may involve life-threatening arrhythmias. Cardiac progenitor cells (CPCs) appear best suited for reconstituting lost myocardium without posing arrhythmic risks, being commissioned towards cardiac phenotype. In this study we tested the hypothesis that mobilization of CPCs through locally delivered Hepatocyte Growth Factor and Insulin-Like Growth Factor-1 to heal chronic myocardial infarction (MI), lowers the proneness to arrhythmias. We used 133 adult male Wistar rats either with one-month old MI and treated with growth factors (GFs, n = 60) or vehicle (V, n = 55), or sham operated (n = 18). In selected groups of animals, prior to and two weeks after GF/V delivery, we evaluated stress-induced ventricular arrhythmias by telemetry-ECG, cardiac mechanics by echocardiography, and ventricular excitability, conduction velocity and refractoriness by epicardial multiple-lead recording. Invasive hemodynamic measurements were performed before sacrifice and eventually the hearts were subjected to anatomical, morphometric, immunohistochemical, and molecular biology analyses. When compared with untreated MI, GFs decreased stress-induced arrhythmias and concurrently prolonged the effective refractory period (ERP) without affecting neither the duration of ventricular repolarization, as suggested by measurements of QTc interval and mRNA levels for K-channel α-subunits Kv4.2 and Kv4.3, nor the dispersion of refractoriness. Further, markers of cardiomyocyte reactive hypertrophy, including mRNA levels for K-channel α-subunit Kv1.4 and ß-subunit KChIP2, interstitial fibrosis and negative structural remodeling were significantly reduced in peri-infarcted/remote ventricular myocardium. Finally, analyses of BrdU incorporation and distribution of connexin43 and N-cadherin indicated that cytokines generated new vessels and electromechanically-connected myocytes and abolished the correlation of infarct size with deterioration of mechanical function. In conclusion, local injection of GFs ameliorates electromechanical competence in chronic MI. Reduced arrhythmogenesis is attributable to prolongation of ERP resulting from improved intercellular coupling via increased expression of connexin43, and attenuation of unfavorable remodeling.

Transcatheter closure of patent ductus arteriosus: experience with a new device.

Transcatheter closure is the preferred method of treatment of patent ductus arteriosus (PDA). Detachable coils are widely used to close small ducts, while the Amplatzer duct occluder (ADO) is generally employed for moderate or large ducts. Recently a new device, the Amplatzer duct occluder II (ADO II), a nitinol flexible mesh, with a symmetrical design to provide high conformability for treatment of all types of PDA, has received the European Community mark approval. We report on one of the first experience, four cases (1 male, 3 female, age ranging from six months to seven years old) with different type and size of PDA treated with the new device. The use of this new Amplatzer duct occluder in our experience has the advantage of ease and safety of placement, conformability, stability, low profile catheters, adaptability for long ducts as in type E.

Acute myocardial infarction in a child: possible pathogenic role of patent foramen ovale associated with heritable thrombophilia.

We report an 8-year-old girl who presented with clinical features of an acute myocardial infarction. The angiographic appearance of the coronary arteries was normal. A thrombophilic state caused by a homozygote genotype for the prothrombin G20210A mutation was detected, and a patent foramen ovale (PFO) with right-to-left shunting after Valsalva maneuver was demonstrated by transesophageal contrast echocardiography. No other embolic source was identified. We suggest that paradoxical embolization through a PFO resulted in a myocardial infarction in this young patient with hereditary thrombophilia. We closed the patient's PFO with a 25-mm PFO occluder. She was anticoagulated with warfarin for 6 months. After 6 months, a contrast echocardiogram showed no evidence of residual atrial shunt. There has been no evident recurrent paradoxical embolization.

Descending thoracic aorta to left pulmonary artery fistula after stent implantation for acquired left pulmonary artery stenosis.

This is a case report of a Fontan patient with previous Potts anastomosis who underwent stent implantation for left pulmonary artery stenosis. At follow-up the patient developed a fistula between the descending thoracic aorta and the left pulmonary artery at the site of the stent. This represents a late complication of stent placement.