Nicola Carano

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).

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.

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

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 …

An equivalent posture to squatting is seen in an unoperated adult with tetralogy of Fallot.

Sir, The advances in paediatric cardiology and cardiac surgery have led to earlier and earlier surgical repair of congenital cardiac malformations. In consequence, the clinical picture of many congenital cardiac malformations has changed, and some signs and symptoms are no longer observed in their typical appearance. Sometimes, however, it is possible to recognise the transformation of an old symptom or sign into a new one, with a new clinical appearance, as we have recently observed in an adult patient with tetralogy of Fallot. The old sign is squatting, which is the characteristic instinctive knees-to-chest-position assumed after exertion by patients with certain types of cyanotic congenital cardiac malformations, notably tetralogy of Fallot. Squatting was initially brought to prominence by Taussig, and generally appears at about one year of age, or when the child starts walking. Investigations have shown that the saturations of oxygen, diminished by exercise, can return to the levels prior to exercise more rapidly when the subject adopts a squatting rather than a standing position. This beneficial effect of squatting is probably due to two phenomenons. The first is the exclusion from the circulation of the highly unsaturated blood from the legs, due to the compression of the femoral vessels. The second is the reduction in the degree of right-to-left shunting due to the increase of peripheral vascular resistance, which opposes the right-to-left shunt through the ventricular septal defect, and increases the flow of blood to the lungs. During hypoxic spells, an infant incapable of standing also tries to obtain the same physiological effects of squatting, compressing his abdomen against the shoulder of his caregiver. What happens in adults? We have recently observed a 40 year-old man with tetralogy of Fallot, who had always refused surgical treatment. His clinical condition progressively worsened during his life, with increasing cyanosis, dyspnoea on exertion, and severe limitation in his physical activity. This man previously loved working in the countryside, and would not abandon this hobby even if it caused him severe fatigue and cyanosis. When he was exhausted, he would return home on his tractor, and press his abdomen against the top of a small refrigerator that was in the garage, assuming a position with his legs on one side of the refrigerator and his head on the other. This position permitted him more rapidly to return to his usual state more rapidly. This clinical observation indicates that adults with tetralogy of Fallot use manoeuvres that are equivalent to squatting.