scholarly journals Is there a transcatheter solution for a sick neonate with hypoplastic right heart syndrome?: Pulmonary valve perforation in a neonate with hypoplastic right ventricle with pulmonary atresia, restrictive VSD—a case report

2020 ◽  
Vol 72 (1) ◽  
Author(s):  
Parag Barwad ◽  
Krishna Prasad ◽  
Jyothi Vijay ◽  
Sanjeev Naganur
Keyword(s):  
Blood Flow ◽  
Pulmonary Circulation ◽  
Pulmonary Blood Flow ◽  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Pulmonary Regurgitation ◽  
Surgical Techniques ◽  
Pediatric Emergency ◽  
Right Heart ◽  
Palliative Procedure

Abstract Background Hypoplastic right heart syndrome with pulmonary atresia is a rare cyanotic heart disease with poor prognosis requiring urgent intervention to establish the pulmonary blood flow. Pulmonary blood flow is achieved by BT shunt or percutaneous techniques like PDA stenting or pulmonary valve perforation. Various series have shown that early surgical intervention causes high mortality in these patients. Pulmonary valve perforation is a suitable, physiological alternative to surgical techniques in selected patients. Case presentation We report a case of hypoplastic right heart syndrome with pulmonary atresia and restrictive VSD presenting with cyanosis from birth and underwent pulmonary valve perforation successfully. Conclusion Duct-dependent pulmonary circulation is a pediatric emergency, palliative procedure for establishing adequate pulmonary blood flow is essential early in the life. In the management of duct-dependent pulmonary circulation, RVOT perforation is an effective and safe option in suitable high-risk subgroups. The induced pulmonary regurgitation along with established physiological antegrade flow would be beneficial in the remodeling of tripartite/hypertrophied small RV.

scholarly journals The V̇e/V̇co2 slope: a useful tool to evaluate the physiological status of children with congenital heart disease

2020 ◽  
Vol 129 (5) ◽  
pp. 1102-1110
Author(s):  
Arthur Gavotto ◽  
Helena Huguet ◽  
Marie-Christine Picot ◽  
Sophie Guillaumont ◽  
Stefan Matecki ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Pulmonary Atresia ◽  
Pulmonary Regurgitation ◽  
Physiological Status ◽  
Right Heart ◽  
Flow Maldistribution ◽  
Residual Lesions

Using V̇e/V̇co2 slope is useful for children with congenital heart disease. V̇e/V̇co2 slope is sensitive to pulmonary blood flow maldistribution during exercise, this concerns congenital heart disease with pulmonary regurgitation, tricuspid regurgitation, right ventricular hypertension, and right ventricle outflow tract obstacle. V̇e/V̇co2 slope is a good parameter to follow single ventricles and right heart residual lesions (tetralogy of Fallot; pulmonary atresia; truncus arteriosus…).

Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period

1992 ◽  
Vol 2 (4) ◽  
pp. 377-381 ◽  
Author(s):  
Michael L. Rigby ◽  
Micelia Salgado ◽  
Celia Silva
Keyword(s):  
Blood Flow ◽  
Right Ventricle ◽  
Pulmonary Blood Flow ◽  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Cross Sectional ◽  
Mitral Valves ◽  
Significant Difference ◽  
The Right ◽  
Pulmonary Valvar Stenosis

SummaryA retrospective study was undertaken of patients with hypoplastic right ventricles, either with pulmonary atresia and intact ventricular septum or critical pulmonary valvar stenosis, and duct-dependent pulmonary blood flow who were investigated at the Royal Brompton Hospital between January 1976 and December 1990. The diagnosis was made on the basis of at least one diagnostic method (cross-sectional echocardiography, cardiac catheterization and angiography, or autopsy). Of the patients, 56 (82%) were found to have an imperforate pulmonary valve or infundibulum (pulmonary atresia), while 12(18%) had critical pulmonary valvar stenosis. The ratio of the diameters of the tricuspid and mitral valvar orifices was measured angiographically during diastole, and the right ventricle was analyzed according to the presence or overgrowth of the inlet, apical trabecular and outlet components. A correlation was made between the severity of the disease and the outcome. The overall mortality was 53% when those not undergoing any surgery were excluded, and significant differences were found between the group dying and those who survived. The incremental risk factors for death were a ratio between the diameter of the tricuspid and mitral valves of less than 0.6; the presence of fistulous communications with the coronary arteries; and obliteration of the apical trabecular component of the right ventricle. There was no significant difference between the group with pulmonary valvar atresia and the group with critical stenosis of the pulmonary valve.

Patent Ductus Arteriosus Banding for Circular Shunting After Pulmonary Valvuloplasty

2016 ◽  
Vol 8 (5) ◽  
pp. 643-645 ◽  
Author(s):  
Carles Bautista-Rodriguez ◽  
Javier Rodriguez-Fanjul ◽  
Julio Moreno Hernando ◽  
Javier Mayol ◽  
Jose Maria Caffarena-Calvar
Keyword(s):  
Blood Flow ◽  
Patent Ductus Arteriosus ◽  
Pulmonary Blood Flow ◽  
Pulmonary Valve ◽  
Ductus Arteriosus ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
Increased Blood Flow ◽  
Critical Pulmonary Stenosis ◽  
Patent Ductus

We report two cases of newborns with critical pulmonary stenosis having intact ventricular septum, who underwent pulmonary valve balloon valvuloplasty followed by banding of a patent ductus arteriosus. Transcatheter pulmonary valvuloplasty was performed one week after delivery. Following the procedure, both developed “circular shunting” as a consequence of left-to-right ductal flow and pulmonary regurgitation. This in turn caused increased blood flow into a dysfunctional right ventricle and low systemic cardiac output syndrome. The PDA banding was performed urgently as a rescue measure in order to restore systemic flow while still maintaining some duct-dependent pulmonary blood flow. This approach resolved the circular shunting. Outcome was favorable in both the patients.

CLINICAL CONFERENCE

PEDIATRICS ◽  
1957 ◽  
Vol 19 (6) ◽  
pp. 1139-1147
Author(s):  
Mary Allen Engle
Keyword(s):  
Blood Flow ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Pulmonary Blood Flow ◽  
Ventricular Hypertrophy ◽  
Pulmonary Valve ◽  
Muscular Tissue ◽  
Pulmonic Stenosis ◽  
Infundibular Stenosis ◽  
The Right

Dr. Engle: When pulmonic stenosis occurs as an isolated congenital malformation of the heart, it usually is due to fusion of the valve cusps into a dome with a small hole in the center. In Figure 1 the pulmonary artery has been laid open so that one can see the three leaflets of the pulmonary valve are completely fused, and that there is only a small, central, pinpoint opening which permits blood to leave the right ventricle and enter the pulmonary circulation. Valvular pulmonic stenosis is much more common than subvalvular or infundibular stenosis, where the obstruction to pulmonary blood flow lies within the substance of the right ventricle. There it may be due to a diaphragm of tissue which obstructs the outflow of the right ventricle, or to an elongated narrow tunnel lined with thickened endocardium, or to a ridge of fibrous or muscular tissue just beneath the pulmonary valve. The changes in the cardiovascular system which result from obstructed pulmonary blood flow are so characteristic that they permit the ready recognition of this condition. Proximal to the constriction, these changes manifest the burden placed on the right ventricle, which enlarges and hypertrophies. On physical examination this is demonstrated by the precordial bulge and tapping impulse just to the left of the sternum, where the rib cage overlies the anterior (right) ventricle. Radiographically, both by fluoroscopy and in roentgenograms in the frontal and both oblique views, right ventricular enlargement is seen. In the electrocardiogram, the precordial leads show a pattern of right ventricular hypertrophy.

Influence of pulmonary blood flow on management and outcome

2011 ◽  
Keyword(s):  
Blood Flow ◽  
Heart Disease ◽  
Pulmonary Circulation ◽  
Early Life ◽  
Pulmonary Blood Flow ◽  
Venous Blood ◽  
Cyanotic Heart Disease ◽  
Capillary Membrane ◽  
Pulmonary Vascular Development ◽  
Alveolar Capillary

Introduction 50Pulmonary vascular development in early life 50Cyanotic heart disease and pulmonary blood flow 52Delivery of systemic venous blood to the alveolar capillary membrane to allow release of waste CO2 and uptake of O2 depends on the integrity of the pulmonary circulation. Too little blood flow to the lungs and the patient is hypoxic; too much and the lungs become oedematous....

In Vitro Study of Flow Regulation for Pulmonary Insufficiency Using Diode Valves

2007 ◽  
Author(s):  
Tiffany A. Camp ◽  
Stephanie Hequembourg ◽  
Richard S. Figliola ◽  
Tim McQuinn
Keyword(s):  
Pulmonary Valve ◽  
Pulmonary Regurgitation ◽  
Circulatory System ◽  
In Vitro Study ◽  
Pulmonary Insufficiency ◽  
Pressure Gradients ◽  
Right Heart ◽  
Mock Circulatory System ◽  
The Right

The operating pressures in the right heart are significantly lower than those of the left heart and with marked differences in the circulation impedances. The pulmonary circulation shows a tolerance for mild regurgitation and pressure gradient [1]. Pulmonary regurgitation fractions on the order of 20% and transvalvular pressure gradients of less than 25mm Hg are considered mild [4]. Given this tolerance, we examine the concept of using a motionless valve to regulate flow in the pulmonary position. In a previous study, the use of fluid diodes was shown to be a promising concept for use as a pulmonary valve [2]. In this study, we test two different diode designs. For each diode valve, flow performance was documented as a function of pulmonary vascular resistance (PVR) and compliance. Tests were done using a pulmonary mock circulatory system [3] over the normal adult range of PVR and compliance settings.

Increasing oxygen tension dilates fetal pulmonary circulation via endothelium-derived relaxing factor

1993 ◽  
Vol 265 (1) ◽  
pp. H376-H380 ◽  
Author(s):  
M. H. Tiktinsky ◽  
F. C. Morin
Keyword(s):  
Blood Flow ◽  
Oxygen Tension ◽  
Hyperbaric Oxygen ◽  
Pulmonary Circulation ◽  
Left Atrial ◽  
Pulmonary Blood Flow ◽  
Absolute Pressure ◽  
Relaxing Factor ◽  
Pulmonary Arterial ◽  
Endothelium Derived Relaxing Factor

We examined the role of endothelium-derived relaxing factor (EDRF) in the increase in pulmonary blood flow caused by increasing oxygen tension in the lungs of the fetus. Fetal lambs at 133 days of gestation were instrumented for intrauterine measurement of pulmonary arterial, left atrial, and amniotic fluid pressure and pulmonary blood flow. Three days later oxygen tension in the pulmonary arterial blood of the fetus was doubled by having the ewe breathe 100% oxygen at 3 atm absolute pressure. In the control fetuses (n = 5), hyperbaric oxygenation increased pulmonary blood flow eightfold. Blocking EDRF production by infusing 45 mg of NG-monomethyl-L-arginine into the superior vena cava of the fetus over 5 min starting 30 min after the beginning of hyperbaric oxygen reversed the increase in pulmonary blood flow (n = 5). Blocking EDRF production by infusing NG-nitro-L-arginine at 1 mg/min for 60 min starting 30 min before hyperbaric oxygen blunted the initial increase in pulmonary blood flow and eliminated it by the end of the experiment (n = 5). As hyperbaric oxygen did not significantly alter pulmonary arterial or left atrial pressure, changes in pulmonary vascular conductance paralleled those in pulmonary blood flow. We conclude that the majority of the vasodilation of the fetal pulmonary circulation caused by increasing oxygen tension is mediated by EDRF. We speculate that EDRF is involved in maintaining low vascular tone at the relatively high oxygen tension of the postnatal lung.

Leukotriene end organ antagonists increase pulmonary blood flow in fetal lambs

1985 ◽  
Vol 249 (3) ◽  
pp. H570-H576 ◽  
Author(s):  
S. J. Soifer ◽  
R. D. Loitz ◽  
C. Roman ◽  
M. A. Heymann
Keyword(s):  
Heart Rate ◽  
Blood Flow ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Pulmonary Circulation ◽  
Pulmonary Blood Flow ◽  
Blood Gases ◽  
Physiological Control ◽  
Arterial Blood ◽  
High Pulmonary Vascular Resistance

The factors responsible for maintaining the normally low pulmonary blood flow and high pulmonary vascular resistance in the fetus are not well understood. Since leukotrienes are potent pulmonary vasoconstrictors in many adult animal species, we determined whether leukotrienes were perhaps involved in the control of the fetal pulmonary circulation by studying the effects of putative leukotriene end organ antagonists in two groups of fetal lambs. In six fetal lambs studied at 130-134 days gestation, FPL 55712 increased pulmonary blood flow by 61% (P less than 0.05) and reduced pulmonary vascular resistance by 45% (P less than 0.05). There was a small increase in heart rate but no changes in pulmonary and systemic arterial pressures and systemic arterial blood gases. In six other fetal lambs studied at 130-140 days gestation, FPL 57231 increased pulmonary blood flow by 580% (P less than 0.05) and decreased pulmonary vascular resistance by 87% (P less than 0.05). Pulmonary and systemic arterial pressures decreased (P less than 0.05), and heart rate increased (P less than 0.05). Leukotriene end organ antagonism significantly increases fetal pulmonary blood flow and decreases pulmonary vascular resistance. Leukotrienes may play a role in the physiological control of the fetal pulmonary circulation.

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