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Non-surgical treatment of congenital left ventricle to coronary sinus fistula and Wolf-Parkinson-White

Minocha, Prashant K; Saharan, Sunil; Chun, Anne; Presti, Salvatore; Cecchin, Frank; Argilla, Michael
Congenital left ventricle to coronary sinus fistula is a rare entity. We report a case of an infant with prenatal finding of left ventricle to right atrial shunt. The anatomy was defined by multi-modality imaging. Baseline electrocardiogram was notable for a Wolff-Parkinson-White pattern. He underwent successful catheter device closure of the left ventricle to coronary sinus fistula. The patient developed supraventricular tachycardia and underwent successful ablation of the accessory pathway.
PMID: 35383549
ISSN: 1467-1107
CID: 5201622

Cardiac Screening in Infants with Infantile Hemangiomas before Propranolol Treatment

Blei, Francine; McElhinney, Doff B; Guarini, Ascanio; Presti, Salvatore
There is no uniform pretreatment cardiac evaluation for infants treated with oral propranolol, which is now the drug of choice for hemangiomas of infancy requiring systemic medical intervention. The aim of this study was to report and evaluate the findings of pretreatment cardiac evaluation. Data were reviewed for patients evaluated by a single hemangioma specialist and a single pediatric cardiologist prior to initiation of propranolol for infantile hemangioma. Cardiac evaluation included a complete echocardiogram. From July 2009 through January 2013, 239 consecutive patients 12 months of age or younger (median 2.7 months) were screened. No patients had cardiac contraindications to propranolol. However, 50 patients (21%) had an abnormal echocardiogram: 39 atrial septal defects (5 associated with right heart enlargement), 6 ventricular septal defects, 2 patent ductus arteriosus, 1 aortic coarctation, 1 pulmonary valve stenosis, and 1 aberrant subclavian artery. Overall, 69 patients had an audible heart murmur, 44 of which were not associated with pathologic findings on echocardiogram. All patients with a ventricular septal defect and 16 of 39 with an atrial septal defect had a murmur. Two of seven patients with PHACE syndrome had cardiac anomalies. None of the findings precluded the use of propranolol. Assisted reproductive technologies were used in 18% of pregnancies, including in vitro fertilization in 12%. Cardiac contraindications to propranolol treatment are uncommon in patients with infantile hemangioma. However, anatomic abnormalities were more common than reported in the general population. Further study is necessary to determine whether there is a pathogenic relationship between cardiac defects and nonsyndromic infantile hemangioma.
PMID: 24889812
ISSN: 0736-8046
CID: 1030822

Vein of Galen malformations in neonates: new management paradigms for improving outcomes

Berenstein, Alex; Fifi, Johanna T; Niimi, Yasunari; Presti, Salvatore; Ortiz, Rafael; Ghatan, Saadi; Rosenn, Barak; Sorscher, Michelle; Molofsky, Walter
BACKGROUND: Untreated patients with symptomatic neonatal presentation of vein of Galen aneurismal malformations (VGAMs) carry almost 100% morbidity and mortality. Medical management and endovascular techniques for neonatal treatment have significantly evolved. OBJECTIVE: To evaluate the clinical and angiographic outcomes of modern management of neonates with refractory heart failure from VGAMs. METHODS: From 2005 to 2010, 16 neonatal patients with VGAM presented to our institution. Medical care from the prenatal to perinatal stages was undertaken according to specified institutional guidelines. Nine patients with refractory heart failure required neonatal endovascular intervention. All patients were treated by transarterial deposition of n-butyl cyanoacrylate into fistula sites. Short- and long-term angiographic studies and clinical outcomes were reviewed. RESULTS: Control of heart failure was achieved in 8 patients. One premature baby died shortly after treatment. Long-term angiographic follow-up shows total or near-total angiographic obliteration in all 8 patients. One patient has a mild hemiparesis from treatment. Another has a mild developmental delay. One patient developed a severe seizure disorder and developmental delay. Overall, 66.7% patients have normal neurological development with near-total or total obliteration of the malformation. CONCLUSION: Treatment of refractory heart failure in neonatal VGAM with modern prenatal, neurointensive, neuroanesthetic, and pediatric neuroendovascular care results in significantly improved outcomes with presumed cure and normal neurological development in most.
PMID: 22089754
ISSN: 0148-396x
CID: 969542

The intermittent ductus revisited: echocardiographic evidence and successful coil occlusion: a case report and review of literature [Case Report]

Verma R; Presti S; Danilowicz D
Intermittent occurrence of a large ductal shunt by physical examination and Doppler echocardiography is reported. Cineangiography confirmed a tubular ductus arteriosus with an angulated, narrow, pulmonary end. Presumably this angulation intermittently caused functional closure of the ductus. Trans-arterial delivery of coils resulted in complete occlusion
PMID: 9829883
ISSN: 0098-6569
CID: 57277

Results of urgent or emergency repair for symptomatic infants under one year of age with single or multiple ventricular septal defect

Danilowicz D; Presti S; Colvin S; Galloway A; Langsner A; Doyle EF
PMID: 1536125
ISSN: 0002-9149
CID: 13683

The disappearing pulmonary artery band [Case Report]

Danilowicz D; Presti S; Colvin S
A child is presented who had pulmonary artery bands that eroded or cut-through to the point where congestive heart failure and pulmonary arterial hypertension recurred. A summary of previously reported cases of band erosion or cut-through is presented, noting that many of these children died. Pulmonary artery banding is now mainly used in infants with complex defects where mortality of early repair is prohibitive or where the Fontan procedure is the only 'repair' possible. To obtain good results, a normal pulmonary vascular resistance is preferred; therefore, it is important that the pulmonary artery band is truly protective over the period of time needed. The occurrence of pulmonary vascular disease can cause a decrease in left-to-right shunting and allow a child to clinically improve, thus duplicating the response to a successful banding. If noninvasive evaluation cannot provide clear-cut proof that the band is protective, then measurement of pulmonary artery pressure and flow must be done
PMID: 2406707
ISSN: 0172-0643
CID: 18063

CARDIAC DISEASE IN CHILDREN WITH HUMAN IMMUNODEFICIENCY VIRUS (HIV) INFECTION [Meeting Abstract]

Rigaud, M; Avnisinger, J; Presti, S; Lawrence, R; Krasinski, K; Borkowsky, W
ISI:A1989T947101116
ISSN: 0031-3998
CID: 31732

Congenital fistulous tract between aorta and right atrium presenting as heart failure in a newborn [Case Report]

Danilowicz D; Presti S; Colvin S; Rutkowski M
A newborn with a fistulous communication between the right coronary sinus and right atrium had congestive heart failure on the first day of life. Medical management was unsuccessful and operative repair was performed using bypass surgery at 5 days of age. Echocardiographic evaluation preoperatively diagnosed the defect correctly and postoperatively confirmed a complete repair. Since aneurysms and/or fistulae can occur in other sinuses after an initial repair of the presenting lesion, the infant will continue to be followed with periodic echocardiographic and clinical evaluations
PMID: 2726603
ISSN: 0172-0643
CID: 10774

Acute systemic embolic events due to mitral valve disease in pediatrics

Friedman, DM; Presti, S; Danilowicz, D; Doyle, EF; Colvin, SB
Three children are presented with systemic embolization occurring from the mitral valve during or following an infectious illness. In all three, normal sinus rhythm was present, and all were able to be treated successfully for the underlying infection, followed by reconstructive valve procedures with annuloplasty. Although systemic embolization related to mitral valve disease is rare in the pediatric age group, these cases emphasize the circumstances under which this can occur, in the absence of complicating dysrhythmias
SCOPUS:0024783909
ISSN: 0885-6265
CID: 584262

Digoxin toxicity in a premature infant: treatment with Fab fragments of digoxin-specific antibodies [Case Report]

Presti S; Friedman D; Saslow J; Doyle EF; Butler VP; Smith TW; Haber E
The first use of Fab fragments to treat digoxin toxicity in a premature infant with renal failure, 18 h after the onset of severe arrhythmias, is reported with dramatic results. The development of digoxin toxicity in the context of accepted therapeutic dosing to treat heart failure due to a cerebral arteriovenous malformation is discussed
PMID: 4059073
ISSN: 0172-0643
CID: 58446