Atenolol Versus Propranolol for Treatment of Infantile Hemangiomas During the Proliferative Phase: A Retrospective Noninferiority Study.

BACKGROUND/OBJECTIVES: The nonselective beta-blocker propranolol is the current criterion standard for treatment of infantile hemangiomas (IHs) and the first therapy that the U.S. Food and Drug Administration has approved for the condition, but concern about adverse effects, such as bronchospasm, hypoglycemia, and sleep disturbances, has sparked interest in the use of alternative agents such as the selective ß1 antagonist atenolol. Our aim was to compare the efficacy and adverse effect profiles of atenolol with those of propranolol in the treatment of IHs in a retrospective noninferiority trial. METHODS: Twenty-seven children with IHs treated with atenolol according to the Cleveland Clinic foundation's standardized clinical assessment and management plan (SCAMP) met inclusion criteria and were compared with a matched group of 53 children with IHs treated with propranolol. Three reviewers assessed response to therapy using a modified version of the previously validated Hemangioma Activity Score (HAS). RESULTS: The mean change in HAS was -2.94 ± 1.20 for patients treated with atenolol and -2.96 ± 1.42 for those treated with propranolol. There was no statistically significant difference in pre- and posttreatment modified HAS scores between the two groups (p = 0.60). There was no significant difference in the overall rate of adverse effects (p = 0.10), although 11% of patients treated with propranolol experienced reactive airway symptoms, whereas this was not seen in any of the patients treated with atenolol. CONCLUSION: Our study supports previous findings that atenolol is at least as effective as propranolol for treatment of IHs and poses less risk of bronchospasm. Our SCAMP proposes guidelines for dosing and monitoring parameters.

Brimonidine Toxicity Secondary to Topical Use for an Ulcerated Hemangioma.

Combigan (Allergan, Irvine, CA) is an ophthalmic solution that combines 0.2% brimonidine, a selective α-2 adrenergic agonist, with 0.5% timolol, a nonselective ß-adrenergic antagonist. It is approved for the reduction of intraocular pressure in patients with glaucoma or ocular hypertension. There have been recent reports of successful treatment of superficial infantile hemangiomas (IHs) using Combigan topically. We report the case of a 2-month-old girl who developed life-threatening brimonidine toxicity requiring intubation and mechanical ventilation secondary to central nervous system depression and apnea after topical application to an ulcerated IH.

Beta Blockade as Treatment for Intracranial Infantile Hemangioma: Case Report and Literature Review.

BACKGROUND: Intracranial infantile hemangiomas are extremely rare, with only 36 patients reported in literature. Treatment for intracranial infantile hemangiomas has been mostly limited to surgery, steroids, and interferon therapy. Propranolol, which is often used to treat cutaneous infantile hemangiomas, is not currently standard treatment for intracranial infantile hemangiomas. PATIENT DESCRIPTION: We present a one-month old boy with an intracranial infantile hemangioma treated with propranolol. RESULTS: This boy was being treated with oral propranolol for a supraclavicular infantile hemangioma. Subsequent brain magnetic resonance imaging (MRI) scan showed evidence of an associated intracranial infantile hemangioma in the right cerebellopontine angle. Repeat brain MRI scan after two months of propranolol treatment demonstrated a significant reduction in the size of the intracranial infantile hemangioma. CONCLUSIONS: This is the first report of successful therapy of an intracranial infantile hemangioma with propranolol.

Propranolol for Treatment of Genital Infantile Hemangioma.

PURPOSE: Genital infantile hemangiomas are vascular anomalies that often require complex management and interdisciplinary care. Propranolol was first used to treat patients with infantile hemangiomas in 2008 and has since gained acceptance as first-line therapy. MATERIALS AND METHODS: We review the presentation, course, management and outcomes of all cases of genital infantile hemangiomas managed by propranolol administration at a single institution from April 2010 to July 2014. RESULTS: During the study period 9 patients with genital infantile hemangiomas were referred to our hemangioma treatment clinic. Propranolol was initially administered under careful outpatient monitoring at a dose of 1 mg/kg daily in 8 patients. One patient, a 700 gm premature infant, was started on therapy in the inpatient setting at 0.5 mg/kg daily, given the history of prematurity. All patients underwent successful increase of dose to at least 2 mg/kg for the observation phase after tolerating the starting doses. One patient discontinued propranolol prematurely per parental request due to concern regarding peripheral vasoconstriction. Otherwise, no patient demonstrated significant hypotension, symptomatic bradycardia, hypoglycemia or other major side effect requiring treatment discontinuation. All patients who continued the treatment protocol had excellent response to therapy. CONCLUSIONS: Propranolol therapy for genital infantile hemangiomas was successfully initiated and the dosage increased in 9 young children without significant side effects and with marked improvement in all patients who continued on treatment. Propranolol is the only Food and Drug Administration approved therapy for treatment of patients with this vascular anomaly and should be considered first-line therapy for genital infantile hemangiomas.

Three-dimensional rotational angiography during percutaneous device closure of fontan fenestration.

The Fontan circulation was originally described as palliation for tricuspid atresia but was subsequently used for a wide variety of single-ventricle physiologies, with the common thread being inability to separate the ventricles for independent use in the systemic and pulmonary circulations. Fontan baffle fenestrations have been created since the late 1980s to assist in maintaining the cardiac output in the presence of increased resistance to flow through the pulmonary circuit. Many institutions continue to use an approach to Fontan circulation that utilizes routine fenestration followed by percutaneous device closure at a later time, whereas other institutions use fenestrations sparingly. We present the case of a five-year-old female with hypoplastic left heart syndrome with a fenestrated Fontan, who underwent three-dimensional rotational angiography at the time of catheterization for percutaneous device closure of the fenestration.

Pentalogy of cantrell: surgical resection of a biventricular diverticulum.

Pentalogy of Cantrell is a rare diagnosis consisting of several midline defects of the sternum, abdominal wall, diaphragm, pericardium, and heart. One of the known features is ventricular diverticulum that can represent a technical challenge surgically. This is a follow-up case report of the successful simultaneous resection of a biventricular diverticulum and omphalocele repair after previous report of repair of complex intracardiac disease in the same patient.

Hybrid palliation of interrupted aortic arch in a high-risk neonate.

We report a case of a high-risk neonate with interrupted aortic arch (IAA) and ventricular septal defect who underwent a successful hybrid palliative procedure using a ductal stent and bilateral branch pulmonary artery banding. This case represents not only a successful use of hybrid approach in high-risk neonates with IAA, but also introduces an alternative and safe access for ductal stent insertion through the right ventricular infundibulum.

Anomalous origin of the left coronary artery from the right pulmonary artery.

Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is a rare congenital anomaly, one which requires a high index of suspicion in order to be diagnosed promptly and managed accurately. ALCAPA from the right branch pulmonary artery (RPA) is a small subset of ALCAPA in general, with only a few reported cases in the world literature. We report two cases of ALCAPA from the RPA: the first case, an infant, presented in severe cardiac failure requiring preoperative extracorporeal membrane oxygenation (ECMO) for stabilization; and the second, a neonate, presented with severe aortic coarctation (CoA). Both patients underwent successful complete surgical repair using a modified surgical technique, and are doing well in midterm follow-up. We describe our operative technique and review the literature of this rare congenital anomaly.

Individualized approach to the surgical treatment of tetralogy of Fallot with pulmonary atresia.

BACKGROUND: Tetralogy of Fallot with pulmonary atresia is a heterogeneous group of defects, characterised by diverse sources of flow of blood to the lungs, which often include multiple systemic-to-pulmonary collateral arteries. Controversy surrounds the optimal method to achieve a biventricular repair with the fewest operations while basing flow to the lungs on the native intrapericardial pulmonary arterial circulation whenever possible. We describe an individualized approach to this group of patients that optimizes these variables. METHODS: Over a consecutive 10-year period, we treated 66 patients presenting with tetralogy of Fallot and pulmonary atresia according to the source of the pulmonary arterial flow. Patients were grouped according to whether the flow of blood to the lungs was derived exclusively from the intrapericardial pulmonary arteries, as seen in 29 patients, exclusively from systemic-to-pulmonary collateral arteries, as in 5 patients, or from both the intrapericardial pulmonary and collateral arteries, as in the remaining 32 patients. We divided the latter group into 9 patients deemed simple, and 23 considered complex, according to whether the pulmonary arterial index was greater than or less than 90 millimetres squared per metre squared, and whether the number of collateral arteries was less than or greater than 2, respectively. RESULTS: We achieved complete biventricular repair in 58 patients (88%), with an overall mortality of 3%. Repair was accomplished in a single stage in all patients without systemic-to-pulmonary collateral arteries, but was staged, with unifocalization, in the patients lacking intrapericardial pulmonary arteries. Complete repair without unifocalization was achieved in all patients with the simple variant of the mixed morphology, and in 56% of patients with the complex variant. The average number of procedures per patient to achieve complete repair was 1, 2.2, 3.8, and 2.6 in patients with exclusively native intrapericardial, simple and mixed, complex and mixed and exclusively collateral pulmonary arterial flow, respectively. CONCLUSIONS: An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.

Coarctation of the aorta: stenting in children and adults.

Coarctation of the aorta may present in infants, children, or adults, and it requires treatment to prevent serious morbidity and mortality. Recent advances in equipment and a growing collective experience have made placement of balloon-expandable stents a safe and effective alternative to surgery or angioplasty in a growing range of patients. This review seeks to provide a working aid for stenting of coarctation of the aorta, based on the techniques and technical considerations in practice at our institution. Between 1989 and 2005, the Congenital Cardiovascular Interventional Study Consortium (CCISC), a consortium of 17 centers, of which our institution is the largest contributor, performed 588 stent placements for coarctation of the aorta. Of the 588 procedures, 580 (98.6%) were successful, as defined by reduction of the gradient to less than 20 mm Hg or increase of the ratio of the diameter of the coarctation area (CoA) to the diameter of the descending aorta (DAo) to at least 0.8. There were a total of 84 complications occurring in 69/588 (11.7%) cases. The most common significant complications were femoral access vessel related 15/588 (2.6%), aneurysm formation 13/588 (2.2%), aortic dissection 9/588 (1.5%), and cerebrovascular accident 6/588 (1.0%). There were two procedure-related deaths (0.3%) recorded in the 16-year period. Many of these significant complications occurred in the same patients. Balloon-expandable stents should be considered a safe and very effective treatment modality in a significant subset of patients with coarctation of the aorta.