Howard Michael Rosenfeld

Children's Hospital & Research Center Oakland · Pediatric Cardiology

Objective Long-term outcomes of patients with mucopolysaccharidosis (MPS) VI treated with galsulfase enzyme replacement therapy (ERT) since infancy were evaluated. Methods The study was a multicenter, prospective evaluation using data from infants with MPS VI generated during a phase 4 study (ASB-008; Clinicaltrials.gov NCT00299000) and clinical d...

Congenital heart disease can be difficult to diagnose in infants because they often present with nonspecific symptoms. Their clinical presentation can resemble that of respiratory infections or sepsis, yet typical treatments for these conditions such as fluid resuscitation may worsen their course. We report a case of an 8-week-old boy who had sever...

Objective To report two cases of cardiac injury following blunt thoracic trauma without external evidence of injury. Design Case report and review of the literature. Setting A 23-bed pediatric intensive care unit in an academic children’s hospital. Interventions Two children presented following significant thoracic trauma without external eviden...

A high tricuspid regurgitant jet velocity (TRV) signifies a risk for or established pulmonary hypertension (PH), which is a serious complication in thalassemia patients. The underlying pathophysiology in thalassemia subgroups and potential biomarkers for early detection and monitoring are not well defined, in particular as they relate to spleen rem...

Background Mitochondrial dysfunction is associated with abnormal cell function, oxidative stress and cell death. A progressive impairment of mitochondrial function and/or increased oxidative damage likely contribute to the pathophysiology of SCD, however little is known about bioenergetics in SCD. While previous work demonstrated mitochondrial dysf...

Characteristic cardiac valve abnormalities and left ventricular hypertrophy are present in untreated patients with mucopolysaccharidosis type VI (MPS VI). Cardiac ultrasound was performed to investigate these findings in subjects during long-term enzyme replacement therapy (ERT) with recombi-nant human arylsulfatase B (rhASB, rhN-acetylgalactosamin...

4752 Background An elevated TRV on Doppler echocardiography (echo) identifies pulmonary hypertension (PH) risk, is common in SCD, and is associated with mortality in adults. An elevated TRV is found in 11–15% of children with SCD, and is associated with decreased exercise capacity and low oxygen saturations. An acute rise in pulmonary presssures c...

Characteristic cardiac valve abnormalities and left ventricular hypertrophy are present in untreated patients with mucopolysaccharidosis type VI (MPS VI). Cardiac ultrasound was performed to investigate these findings in subjects during long-term enzyme replacement therapy (ERT) with recombinant human arylsulfatase B (rhASB, rhN-acetylgalactosamine...

The mucopolysaccharidoses (MPSs) are inherited lysosomal storage disorders caused by the absence of functional enzymes that contribute to the degradation of glycosaminoglycans (GAGs). The progressive systemic deposition of GAGs results in multi-organ system dysfunction that varies with the particular GAG deposited and the specific enzyme mutation(s...

Aortico-left ventricle tunnels are rare congenital anomalies with variable anatomy; however, by definition, they consist of an abnormal connection between the ascending aorta and the left ventricle. Diagnosis is usually by echocardiography, and treatment typically uses a two-patch surgical repair. Herein, we describe a case of an aortico-left ventr...

Pulmonary hypertension (PAH), determined on echocardiogram by a tricuspid jet velocity (TJV)≥2.5m/sec in the absence of diastolic dysfunction is a common complication in patients with beta-thalassemia intermedia (TI) and some patients with thalassemia major (TM). The pathogenesis is thought to be multifactorial, primarily due to increased hemolysis...

The pathogenesis of pulmonary hypertension (PAH), a serious complication in thalassemia, is not well understood. Thromboembolism has been postulated as one of the causative factors; however, there are currently limited specific data on its role. To examine whether increased platelet activation and hypercoagulability are linked to PAH, 25 beta-thala...

Pulmonary hypertension (PHT) is a serious, under diagnosed, potentially fatal complication, reported mostly in non-transfused adult thalassemia intermedia (TI) and fewer thalassemia major (TM) patients. Even a mild PHT can further impair cardiac function in these patients with a frequent left heart disease. Yet, understanding of the pathogenesis, e...

An isolated left common carotid artery (LCA) is an extremely rare condition with only four reported cases. In each case, the isolated carotid artery connects to the right or left pulmonary artery via the ductus arteriosus and the embryologic basis for the abnormalities is believed to reflect an error in the development of the branchial arches. We p...

Rather than perform a difficult and potentially high risk coronary reimplantation in a patient with an aberrant right coronary artery coursing between the aorta and pulmonary artery, the main pulmonary artery was translocated toward the left pulmonary hilum to create additional space between the aortic and pulmonic trunks.

Aortic laceration secondary to Palmaz Stent placement for treatment of superior vena cava syndrome is reported. This potentially life-threatening complication should be considered when rigid balloon expandable stents are used to treat superior vena cava syndrome of benign origin. Cathet. Cardiovasc. Intervent. 49:160-162, 2000.

To investigate the accuracy of immediate postbypass transesophageal echocardiography in the assessment of residual cardiac defects, we compared intraoperative transesophageal echocardiograms with intra/postoperative data in 86 patients, aged 4 days to 30.7 years (median = 1.4 years), at risk for a total of 174 postoperative lesions: right (n = 55)...

This study evaluated our early experience with video-assisted thoracoscopic vascular ring division and compared this approach with division by means of a conventional open thoracotomy. Video-assisted thoracoscopic techniques reduce surgical trauma and have been applied to several adult thoracic procedures; however, pediatric applications have been...

Recent development of prototype pediatric biplane transducers has extended the use of biplane TEE imaging to the neonate. One such TEE probe with 64 imaging elements in each pallet was used perioperatively in 46 infants and children (weight 2.9 to 32 kg) undergoing surgery for complex congenital heart disease. The success rate for passing the trans...

We reviewed pre-closure echocardiograms on all patients undergoing transcatheter atrial septal defect (ASD) closure with the Bard double-umbrella occluder device aided by simultaneous transesophageal echocardiography to determine precatheterization predictors of outcome. Transesophageal echocardiograms were performed on 28 of 132 patients (22%) und...

Aortopulmonary septal defect, interruption of the aortic arch, and anomalous origin of the right pulmonary artery from the aorta comprised a challenging constellation of defects in a 13-day-old neonate, who underwent complete repair without homograft tissue or synthetic graft. After the aorta was separated from the pulmonary artery, the descending...

Although balloon aortic valvuloplasty (BAV) is effective in the acute management of valvar aortic stenosis (AS), sustained benefit of this technique has been shown to vary in differing age groups. The role of BAV in the young adult with congenital, nondegenerative AS is poorly defined. The catheterization results and follow-up echocardiographic dat...

Despite the recent wide applicability of the bidirectional cavopulmonary shunt, there is limited reported experience in performing these shunts in infants 6 months or younger. Before October 1992, 17 consecutive infants aged 4.2 to 6.5 months (median, 6.1 months) underwent bidirectional cavopulmonary shunts. The diagnoses were hypoplastic left hear...