Aldo Agnetti

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.

Congenital pulmonary steal phenomenon associated with tetralogy of Fallot, right aortic arch, and isolation of the left subclavian artery.

Isolation of the left subclavian artery has been reported in cases of tetralogy of Fallot with a right aortic arch. As the isolated left subclavian artery is supplied by the left vertebral artery (in which the blood flow direction is inverted), this anomaly is usually responsible for a congenital subclavian steal phenomenon. In our case the isolated left subclavian artery was connected by a patent ductus arteriosus to the main pulmonary artery. Therefore the left vertebral artery did not supply the distal left subclavian artery but supplied the main pulmonary artery (congenital pulmonary artery steal).

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 β-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.

Device closure of fenestrated atrial septal defects: Use of a single Amplatz atrial septal occluder after balloon atrial septostomy to create a single defect

Successful transcatheter device closure of secundum atrial septal defect has achieved worldwide acceptance with the clinical use of the Amplatz atrial septal occluder. However, device closure of multiple fenestrated secundum atrial septal defects remains a challenge for the interventionist and in some cases may be only effectively closed surgically. This case report of multiple fenestrated atrial septal defects represents a further expansion of technologic efforts to improve the results and simplify the mechanism of device closure of fenestrated defects. When device closure with more than one device seems impractical or disadvantageous, consolidation of multiple defects in a fenestrated fossa ovalis by simple balloon atrial septostomy as demonstrated by this case report provides an easy mechanism to allow implantation of a single large device which will effectively close all of the defects. This approach is similar to the surgical approach for resection of the fenestrated membrane with secondary patch closure of the enlarged defect. Excellent and complete closure of a fenestrated secundum atrial septal defect is illustrated in this case report. Cathet Cardiovasc Intervent 2001;52:203–207.

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.

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.

Extended end-to-end anastomosis with modified reverse subclavian flap angioplasty.

We report a surgical treatment for neonatal aortic coarctatin associated with distal aortic arch hypoplasia. This technique offers the possibility for augmentation of the aortic arch without sacrificing the subclavian artery or using prosthetic patch material. The procedure was successfully performed in 5 patients.

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

BackgroundAcquired 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 presentationWe 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.ConclusionRheumatic 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.

Dilated Form of Endocardial Fibroelastosis as a Result of Deficiency in Respiratory-Chain Complexes I and IV

A 2-month-old girl with poor appetite and failure to thrive was admitted because of pallor, dyspnea, and tachycardia with periodic gallop rhythm. Chest x-ray (Figure 1) showed cardiomegaly, and echocardiography (Figure 2) revealed left ventricular dilation (diastolic diameter, 41.5 mm [normal, <23 mm]), decreased contractility, and mild to moderate mitral valve insufficiency. ECG (Figure 3) showed sinus rhythm with left bundle-branch block and repolarization abnormalities. These findings were compatible with severe dilated cardiomyopathy. Polymerase chain reaction of the serum failed to detect any cardiotropic virus genomes. There was lactic acidosis. On frozen striated muscle, the mitochondrial respiratory chain function was tested and found to have decreased activities of complexes I (NADH coenzyme Q reductase, 9.1 nmol/min per milligram [normal, 13 to 24]) and IV (cytochrome- c oxidase, 103 nmol/min per milligram [normal, 120 to 220]). Therapy with oxygen, digoxin, captopril, and furosemide was initiated. Despite this, her cardiovascular function continued to worsen; she was readmitted at 10 months of age for decompensated heart failure and died 8 days later. Her family history revealed 1 brother who had died suddenly at the age of 3 months (no autopsy was performed). Figure 1. Chest x-ray obtained at age 2 months showing significant enlargement of the cardiac profile. Figure 2. Transthoracic echocardiography performed at the same age displaying severe left ventricular (LV) chamber dilation. RV indicates right ventricle; LA, left atrium; and Ao, aorta. Figure 3. ECG obtained at first admission to the hospital showing left bundle-branch block and repolarization abnormalities. Autopsy confirmed cardiac enlargement with severe left ventricular chamber dilation (Figure 4A); the endocardium was whitish and …

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

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.