No Inflammatory Response Related to Pulmonary Hemodynamics in Children with Systemic to Pulmonary Shunts

Objective. The hypothesis was that the levels of circulating inflammatory mediators are related to the degree of volume and pressure stress on the pulmonary vasculature in children with congenital systemic to pulmonary shunts. Design. Prospective, cross‐sectional study. Setting. Tertiary center covering all pediatric heart surgery and interventions in Norway. Patients. Seventy‐four children, aged 0–12 years, admitted for surgical or interventional treatment of congenital systemic to pulmonary shunts. Outcome Measures. Plasma levels of eight mediators of vascular inflammation and endothelial activation, sampled from different vascular compartments. Results. Patients with the most pronounced pulmonary flow and pressure stress demonstrated no elevation of inflammatory mediator levels when compared with healthy controls. No pulmonary production or uptake of the measured markers was found. Hemodynamic explanatory factors showed weak correlations to the inflammatory marker levels by univariate analysis. Age was the only factor that significantly explained inflammatory response in the multivariate model. The presence of Down syndrome, irrespective of hemodynamic category, was associated with elevated plasma levels of soluble tumor necrosis factor receptor I, when controlling for age. Conclusions. Inflammatory mediators show no significant relationship to pulmonary hemodynamics in children with systemic to pulmonary shunts. Children with Down syndrome may have an increased inflammatory response.     

Hemodynamic Effects of Noninvasive Ventilation in Patients with Venocapillary Pulmonary Hypertension

Background

The hemodynamic effects of noninvasive ventilation with positive pressure in patients with pulmonary hypertension without left ventricular dysfunction are not clearly established.

Objectives

Analyze the impact of increasing airway pressure with continuous positive airway pressure on hemodynamic parameters and, in particular, on cardiac output in patients with variable degrees of pulmonary hypertension.

Methods

The study included 38 patients with pulmonary hypertension caused by mitral stenosis without left ventricular dysfunction or other significant valvulopathy. The hemodynamic state of these patients was analyzed in three conditions: baseline, after continuous positive pressure of 7 cmH₂O and, finally, after pressure of 14 cmH₂O.

Results

The population was composed of predominantly young and female individuals with significant elevation in pulmonary arterial pressure (mean systolic pressure of 57 mmHg). Of all variables analyzed, only the right atrial pressure changed across the analyzed moments (from the baseline condition to the pressure of 14 cmH₂O there was a change from 8 ± 4 mmHg to 11 ± 3 mmHg, respectively, p = 0.031). Even though there was no variation in mean cardiac output, increased values in pulmonary artery pressure were associated with increased cardiac output. There was no harmful effect or other clinical instability associated with use application of airway pressure.

Conclusion

In patients with venocapillary pulmonary hypertension without left ventricular dysfunction, cardiac output response was directly associated with the degree of pulmonary hypertension. The application of noninvasive ventilation did not cause complications directly related to the ventilation systems.     

Treatment of Refractory Pulmonary Arterial Hypertension with Inhaled Epoprostenol in an Infant with Congenital Heart Disease

Epoprostenol is a potent arterial vasodilator, and its administration by inhalation localizes its effects to the pulmonary circulation. In this case report, we describe a 3‐month‐old male patient with significant refractory pulmonary hypertension after pulmonary artery banding and placement of a Blalock–Taussig shunt. This patient continued to have significant hypoxic episodes despite maximal therapy with sedation, alkalinization, sildenafil, and inhaled nitric oxide. After the addition of inhaled epoprostenol, improvements in both clinical response and echocardiography‐based hemodynamics were observed. The case supports a synergistic role among the agents in the treatment of pulmonary arterial hypertension from congenital heart disease.     

Pulmonary Hypertension Associated with Congenital Heart Disease: A Practical Review for the Pediatric Cardiologist

Pulmonary hypertension (PH) is a complex condition with a broad range of etiologies that result in a common outcome—elevated pulmonary arterial pressure. For the pediatric cardiologist, this entity provides a manifest demonstration of the interrelationship of cardiac and pulmonary physiology. A thoughtful approach must be applied to each patient with PH in order to achieve a thorough understanding of the nature of disease and to proceed with individualized therapy in an appropriate and timely manner. This article aims to provide a clinically relevant, practical review of current literature related to PH in patients with congenital heart disease, including the causes, diagnostic considerations, and an overview of management.     

Association Between Hemodynamic Profile,Physical Capacity and Quality of Life in Pulmonary Hypertension

Background

No studies have described and evaluated the association between hemodynamics, physical limitations and quality of life in patients with pulmonary hypertension (PH) without concomitant cardiovascular or respiratory disease.

Objective

To describe the hemodynamic profile, quality of life and physical capacity of patients with PH from groups I and IV and to study the association between these outcomes.

Methods

Cross-sectional study of patients with PH from clinical groups I and IV and functional classes II and III undergoing the following assessments: hemodynamics, exercise tolerance and quality of life.

Results

This study assessed 20 patients with a mean age of 46.8 ± 14.3 years. They had pulmonary capillary wedge pressure of 10.5 ± 3.7 mm Hg, 6-minute walk distance test (6MWDT) of 463 ± 78 m, oxygen consumption at peak exercise of 12.9 ± 4.3 mLO2.kg-1.min-1 and scores of quality of life domains < 60%. There were associations between cardiac index (CI) and ventilatory equivalent for CO2 (r=-0.59, p <0.01), IC and ventilatory equivalent for oxygen (r=-0.49, p<0.05), right atrial pressure (RAP) and ''general health perception'' domain (r=-0.61, p<0.01), RAP and 6MWTD (r=-0.49, p<0.05), pulmonary vascular resistance (PVR) and ''physical functioning'' domain (r=-0.56, p<0.01), PVR and 6MWTD (r=-0.49, p<0.05) and PVR index and physical capacity (r=-0.51, p<0.01).

Conclusion

Patients with PH from groups I and IV and functional classes II and III exhibit a reduction in physical capacity and in the physical and mental components of quality of life. The hemodynamic variables CI, diastolic pulmonary arterial pressure, RAP, PVR and PVR index are associated with exercise tolerance and quality of life domains.     

Fenestrated ASD Closure in a Child with Idiopathic Pulmonary Hypertension and Exercise Desaturation

Pulmonary arterial hypertension is a progressive disorder that may result in right heart failure and death. Atrial level shunts in the presence of pulmonary hypertension may allow right‐to‐left mixing with maintenance of cardiac output and improved survival. However, excessive mixing at the atrial level can cause undue systemic desaturation, increased fatigue and decreased exercise tolerance even in the presence of adequate cardiac output. A 5½‐year‐old was diagnosed with pulmonary hypertension, a large atrial septal defect and right‐to‐left shunting. Medical therapy over an 18‐month period was successful in decreasing pulmonary artery pressure and pulmonary vascular resistance. However, because of the size and position of the intracardiac defect, symptoms of fatigue, and severe systemic desaturation with only minor activities persisted. Fenestrated surgical closure of the defect was thus undertaken to decrease the degree of atrial mixing, but still allow atrial decompression if necessary. Subsequent hemodynamic evaluation has demonstrated continued improvement, and all previous symptoms have resolved. Repeated echocardiography has confirmed patency of the atrial fenestration with left‐to‐right atrial flow.     

Rehabilitation Program in Adult Congenital Heart Disease Patients with Pulmonary Hypertension

Introduction. It is commonly believed that physical activity may have a negative impact on pulmonary hypertension patients. The object of this study is to determine the tolerability of a directed exercise program in congenital heart disease patients with pulmonary hypertension. Methods. Eight congenital heart disease patients with pulmonary hypertension were studied and followed up during a 1-year period. Four of them were enrolled in a 3-month rehabilitation program. Results. No significant changes in analytical data, hand and leg strength, or quality of life were seen at the end of the training program in rehabilitation and nonrehabilitation patients. However, patients in the rehabilitation group improved 6 minutes' walk test minimum hemoglobin oxygen saturation and functional class after ending the training program without having adverse events such as progression of symptoms or heart failure. Conclusions. Cardiopulmonary rehabilitation appears to be a safe intervention in patients with congenital heart disease and pulmonary hypertension.     

Imaging of Extralobar Pulmonary Sequestration in a Patient with Tetralogy of Fallot with Pulmonary Atresia,Ventricular Septal Defect and Multiple Aorto‐pulmonary Collateral Arteries with Multidetector Computed Tomography

Complex pulmonary vascular blood supply is common in patients with tetralogy of Fallot with pulmonary atresia, major systemic to pulmonary collateral arteries and hypoplastic or deficient central pulmonary arteries. An extralobar lung sequestration, which has not been described previously in these patients, was imaged in a 6‐week‐old infant with multidetector computed tomography with sub‐millimeter resolution. Arterial and venous vessels were analyzed using three‐dimensional vascular exploration tools and results were confirmed with cardiac catheterization.