Prostanoids in pediatric pulmonary hypertension: clinical response, time-to-effect, and dose-response.

For pediatric pulmonary arterial hypertension (PAH) patients treated with parenteral prostanoids, response predictors, and the dose-effect relationship are ill defined. We determined the following: (1) which pulmonary vascular hemodynamic variable, after initiating prostanoids, best correlates with a significant clinical response; (2) the time interval after treatment when if no pulmonary hemodynamic improvement has occurred, none is ever likely to; and (3) the relationship between the prostanoid dose and its hemodynamic effects. This is a retrospective cohort study of 31 pediatric patients with Group 1 PAH treated with parenteral prostanoids.

Improving Safety of Intravenous Prostacyclin Administration to Pediatric Patients With Pulmonary Hypertension.

Background: Pulmonary hypertension is a rare, life-threatening disease with limited therapeutic options and no definitive cure. Continuous intravenous prostacyclin therapy is indicated for treatment of severe disease. These medications have a narrow therapeutic index and a brief half-life; therefore, administration errors can be lethal.

Pulmonary Artery Banding in Post-tricuspid Congenital Cardiac Shunting Defects with High Pulmonary Vascular Resistance.

Reports of "treat and repair" of cardiac shunting lesions with inoperably high pulmonary vascular resistance (PVR) mostly concern pre-tricuspid defects; post-tricuspid lesions are different. We report our experience with pulmonary artery (PA) banding ± targeted pulmonary hypertension medications in five patients with a large VSD and inoperably high PVR, and review previous reports of PA banding with post-tricuspid defects. Three of our 5 patients had mean PAP > 50 mmHg after banding and no or only a transient fall in PVR.

Systemic Ventricular Dysfunction Between Stage One and Stage Two Palliation.

Infants with a single ventricle can develop systemic ventricular dysfunction (SVD) after stage 1 operation, but available information is sparse. We reviewed our patients having Norwood, Sano, or hybrid procedures to better understand this problem. We conducted a retrospective, case-controlled cohort study of 267 patients having stage1 operation, examining outcomes between stages 1 and 2 (survival and subsequent cardiac surgeries), predictor variables, and histology of hearts explanted at transplantation.

The impact of right ventricular pressure and function on survival in patients with pulmonary vein stenosis.

Pulmonary vein stenosis (PVS) is associated with pulmonary hypertension (PH), but there is little information regarding the impact of PH on right ventricular (RV) systolic function and survival. We conducted a retrospective cohort study of our patients to explore this and other aspects of pulmonary hemodynamics with PVS. RV function was assessed using qualitative two-dimensional echocardiography.

Pulmonary hypertension's variegated landscape: a snapshot.

The many types of pulmonary hypertension (PH) are so protean in their biological origin, histological expression, and natural history that it is difficult to create a summary picture of the disease, or to easily compare and contrast characteristics of one type of PH with another. For newcomers to the field, however, such a picture would facilitate a broad understanding of PH. In this paper, we suggest that four characteristics are fundamental to describing the nature of various types of PH, and that taken together they define a number of patterns of PH expression.

Duration of Mechanical Ventilation and Perioperative Care Quality After Neonatal Cardiac Operations.

Background: This study was conducted to determine whether the duration of mechanical ventilation (DOMV) could be used to benchmark the overall quality of care after neonatal congenital heart operations.

Methods: Children aged younger than 30 days undergoing cardiac operations were reviewed. Technical adequacy was assessed using the Technical Performance Score (TPS), a previously validated tool for determining the adequacy of a palliative or corrective surgical procedure that uses echocardiography criteria and need for unplanned reintervention to determine technical adequacy.

Association of Extracorporeal Membrane Oxygenation Support Adequacy and Residual Lesions With Outcomes in Neonates Supported After Cardiac Surgery.

Objectives: There is a paucity of data regarding the impact of extracorporeal membrane oxygenation support, adequacy of surgical repair, and timing of intervention for residual structural lesions in neonates cannulated to extracorporeal membrane oxygenation after cardiac surgery. Our goal was to determine how these factors were associated with survival.

Design: Retrospective study.

Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society.

Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension.

Lung Pathology in Pediatric Pulmonary Vein Stenosis.

Pulmonary vein stenosis is a rare progressive narrowing of the extrapulmonary pulmonary veins, presenting predominantly in infancy and virtually always lethal. It typically arises following repair of congenital heart disease, particularly anomalous pulmonary venous return. Histologic characterization of pediatric pulmonary vein stenosis, not previously well described, may provide insight into the disease pathobiology.

Central venous line complications with chronic ambulatory infusion of prostacyclin analogues in pediatric patients with pulmonary arterial hypertension.

Chronic infusion of prostacyclin (PGI2) via a Broviac central venous line (CVL) is attended by risk of CVL-related complications, but we know of only one report regarding CVL-associated bloodstream infection (BSI) with PGI2 in children and none regarding other complications. We conducted a retrospective cohort study involving pediatric patients with pulmonary hypertension treated with chronic intravenous infusion of PGI2 at Boston Children's Hospital and determined the rate (per 1,000 line-days) of various CVL-related complications. We also determined how often complications necessitated line replacement and hospitalization, time to replacement of CVLs, and interpatient variability in the incidence of complications.

Pulmonary hypertension caused by pulmonary venous hypertension.

The effect of pulmonary venous hypertension (PVH) on the pulmonary circulation is extraordinarily variable, ranging from no impact on pulmonary vascular resistance (PVR) to a marked increase. The reasons for this are unknown. Both acutely reversible pulmonary vasoconstriction and pathological remodeling (especially medial hypertrophy and intimal hyperplasia) account for increased PVR when present.

Parental perspectives on suffering and quality of life at end-of-life in children with advanced heart disease: an exploratory study*.

Objective: To describe parent perspectives regarding the end-of-life experience of children with advanced heart disease.

Design: Cross-sectional multicenter survey study of bereaved parents.

Setting: Two tertiary care pediatric hospitals.

"Nocturnal seizures" in idiopathic pulmonary arterial hypertension.

The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made.

Quality of life and parental adjustment in pediatric pulmonary hypertension.

Background: This study examines the impact of pulmonary hypertension (PH) on the quality of life (QoL) of affected youth, as well as the relationships among PH disease severity, parental adjustment variables, and family relational functioning.

Methods: Subjects were 47 eligible parents of children with PH aged 2 to 18 years who were evaluated at Boston Children's Hospital. Measures of patient QoL and of parental stress, coping, and adjustment were administered to the caregivers.

Implications of the U.S. Food and Drug Administration warning against the use of sildenafil for the treatment of pediatric pulmonary hypertension.

Pulmonary arterial hypertension (PAH) contributes to disability and death in children with diverse cardiac, pulmonary, or systemic diseases, and therapeutic options are currently limited. Data from adult studies provide the basis for most PAH-specific therapies; however, many of these medications are commonly used in children on an off-label basis due to the life-threatening nature of PAH. Although currently approved for use in adult PAH, sildenafil is used extensively off-label for the treatment of neonates, infants, and children with PAH.

Pulmonary blood flow and pulmonary hypertension: Is the pulmonary circulation flowophobic or flowophilic?

Increased pulmonary blood flow (PBF) is widely thought to provoke pulmonary vascular obstructive disease (PVO), but the impact of wall shear stress in the lung is actually poorly defined. We examined information from patients having cardiac lesions which impact the pulmonary circulation in distinct ways, as well as experimental studies, asking how altered hemodynamics impact the risk of developing PVO. Our results are as follows: (1) with atrial septal defect (ASD; increased PBF but low PAP), shear stress may be increased but there is little tendency to develop PVO; (2) with normal PBF but increased pulmonary vascular resistance (PVR; mitral valve disease) shear stress may also be increased but risk of PVO still low; (3) with high PVR and PBF (e.

Dynamic evolution of practice guidelines: analysis of deviations from assessment and management plans.

Objective: A Standardized Clinical Assessment and Management Plan (SCAMP) standardizes the care of patients with a predefined diagnosis while actively inviting and collecting data on clinician deviations (DEVs) from its recommendations. For 3 different pediatric cardiac diagnoses managed by SCAMPs, we determined the frequency of, types of, and reasons for DEVs, which are considered to be a valuable source of information and innovation.

Methods: DEVs were collected as part of SCAMP implementation.

The impact of pulmonary venous hypertension on the pulmonary circulation in the young.

Objective And Design: Pulmonary venous hypertension is a well-characterized cause of pulmonary hypertension in adults, but little is known regarding the relationship between left atrial pressure and pulmonary arteriolar resistance in the young. Also, in adults relief of pulmonary venous hypertension results in a marked fall in pulmonary arteriolar resistance, but this could be different in children because vascular changes are more severe in young patients than adults with mitral stenosis. We inspected records of children at Children's Hospital Boston having mitral balloon valvuloplasty, and patients ≤5 years old having mitral valve replacement, to determine (1) the relationship between left atrial pressure and pulmonary arterial pressure and resistance (n = 94 children, median age 17.

Pulmonary arterial hypertension: what the large pulmonary arteries tell us.

The morphology of the large intrapulmonary arteries (PAs) in pulmonary hypertension (PH) has received limited attention. Dilation, pruning, abrupt tapering, and tortuosity of PAs occur, but whether different patients have distinct PA phenotypes is unknown. Pulmonary arteriograms from 41 pediatric patients with PH were blindly reviewed by four experts who assigned each angiogram one of three designations: straight (S), tortuous (T), or ambiguous (A).