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

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.

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CLINICAL CONFERENCE

PEDIATRICS ◽

10.1542/peds.19.6.1139 ◽

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.

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Pulmonary atresia with intact ventricular septum in the fetus

Cardiology in the Young ◽

10.1017/s1047951100007964 ◽

1992 ◽

Vol 2 (4) ◽

pp. 367-376 ◽

Cited By ~ 17

Author(s):

Lindsey D. Allan ◽

Andrew Cook

Keyword(s):

Right Ventricle ◽

Poor Prognosis ◽

Pulmonary Atresia ◽

Ventricular Septum ◽

Fetal Life ◽

Intact Ventricular Septum ◽

Cross Sectional ◽

The One ◽

The Right ◽

Echocardiographic Findings

AbstractThe objective of this study was to review the echocardiographic findings and autopsy correlates of pulmonary atresia with an intact ventricular septum when diagnosed during fetal life. All cases with this lesion (nearly 60) seen in the Perinatal Cardiology unit of Guy's Hospital since 1983 are part of this study. More than half of the cases were detected within the last two years, and all bar three were referred because of suspicion of a cardiac malformation detected on routine obstetric scanning. The cases were divided into two distinct groups. On the one hand were those with dilatation of the chambers of the right heart. These had a uniformly poor prognosis. Severe regurgitation of the tricuspid valve was a characteristic feature. On the other hand were the cases with cavitary hypoplasia due to mural overgrowth. Direct measurements of pressure in one such case revealed suprasystemic values in the right ventricle. Although contemplated, fetal intervention proved impossible. Autopsy revealed cases with either valvar or infundibular atresia. In most cases diagnosed prior to 24 weeks of gestation, mothers opted for termination of pregnancy. All cases with dilatation of the right ventricle died. Of pregnancies which continued, only 30% of children are still alive, all but one being less than two years of age at present. The use of cross-sectional and Doppler echocardiography permits the accurate diagnosis of pulmonary atresia with an intact ventricular septum during fetal life. Autopsied cases showed strong correlation with the echocardiographic findings. The poor prognosis in most cases warrants consideration in the future of intervention during fetal life.

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Transient Disturbance of Pulmonary Blood Flow and Subsequent Pressure Overloading in the Right Ventricle Following the Inhalation of a Water-proof Spray

Journal of the Japanese Society of Intensive Care Medicine ◽

10.3918/jsicm.3.99 ◽

1996 ◽

Vol 3 (2) ◽

pp. 99-102 ◽

Cited By ~ 2

Author(s):

Noriaki Aoki ◽

Takashi Masuda ◽

Kazui Soma ◽

Toshiro Kurosawa ◽

Takashi Ohwada

Keyword(s):

Blood Flow ◽

Right Ventricle ◽

Pulmonary Blood Flow ◽

The Right ◽

Water Proof

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Natural history of congenital pulmonary valvar stenosis: an echo and Doppler cardiographic study

Cardiology in the Young ◽

10.1017/s1047951100008337 ◽

1999 ◽

Vol 9 (2) ◽

pp. 129-135 ◽

Cited By ~ 18

Author(s):

Hanneke Gielen ◽

Otto Daniëls ◽

Henk van Lier

Keyword(s):

Right Ventricle ◽

Pressure Gradient ◽

Pulmonary Valve ◽

Anterior Wall ◽

Jet Stream ◽

Significant Relation ◽

Severe Stenosis ◽

History Of ◽

The Right ◽

Pulmonary Valvar Stenosis

AbstractDoppler echocardiography allows accurate serial assessment of pulmonary valvar stenosis by measuring the velocity of the jet stream through the pulmonary valve. Between 1979 and 1997, we saw 174 patients with isolated pulmonary valvar stenosis. At admission their ages ranged from 9 days to 22.5 years. We measured the velocity over the pulmonary valve, and the thickness of the anterior wall of the right ventricle, and made a study of their electrocardiograms. We found that rapid increases and decreases occurred in almost every age-group. For patients with a trivial, mild or moderate level of stenosis, severe stenosis developed in 3, 10 and 9%, respectively. In most of the patients, 122 (90%), in whom there was more than one examination, a change in pressure gradient between −12mmHg/year and +3mmHg/year was found. Only 7 patients had an increase of more than 10mmhg per year. In contrast with our patients having aortic stenosis, these with stenosis of the pulmonary valve showed no rapid increase in early childhood. Indeed, in 58% the severity of the stenosis decreased. No correlation was found when comparing the echocardiographic measurements of the thickness of the anterior wall of the right ventricle with the voltages on the electrocardiogram. A significant relation was found however, between an increasing pressure gradient and thickened valvar leaflets (p = 0.017).

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Effect of pulmonary blood flow on leukocyte uptake and release by dog lung

Journal of Applied Physiology ◽

10.1152/jappl.1984.56.4.966 ◽

1984 ◽

Vol 56 (4) ◽

pp. 966-974 ◽

Cited By ~ 31

Author(s):

H. V. Thommasen ◽

B. A. Martin ◽

B. R. Wiggs ◽

M. Quiroga ◽

E. M. Baile ◽

...

Keyword(s):

Blood Flow ◽

Right Ventricle ◽

Pulmonary Blood Flow ◽

Recovery Period ◽

Control Period ◽

Balloon Catheter ◽

Vena Cava ◽

Low Flow ◽

The Right ◽

Uptake And Release

The effect of pulmonary blood flow on leukocyte uptake and release by the lung was examined in 10 anesthetized spontaneously breathing dogs. Pulmonary arterial and pulmonary venous blood was sampled with catheters placed into the right ventricle and aorta, respectively. Pulmonary blood flow was lowered by inflating a balloon catheter located in the inferior vena cava. In five experiments simultaneous blood samples were drawn from the right ventricle and aorta at 10-s intervals during a control period, a 2- to 3-min period of low flow, and a recovery period. In five additional experiments, less frequent samples were taken over periods of 15–60 min. Total leukocyte concentrations and differential counts were determined for each blood sample. The study shows that large numbers of leukocytes become sequestered within the lung when pulmonary blood flow is low and that an equivalent number of cells are released from the lung after deflation of the balloon catheter. Both the polymorphonuclear leukocytes and the lymphocytes were taken up by the lung when pulmonary blood flow was reduced. We conclude that pulmonary blood flow has a marked effect on the uptake and release of leukocytes by the dog lung.

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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

The Egyptian Heart Journal ◽

10.1186/s43044-020-00097-7 ◽

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.

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CLOSING OF INTERVENTRICULAR SEPTUM DEFECT IN ONE-STEP AND STEPWISE SURGICAL TREATMENT IN PATIENTS WITH PULMONARY ATRESIA AND AORTO-PULMONARY COLLATERALS

Grekov s Bulletin of Surgery ◽

10.24884/0042-4625-2015-174-4-9-12 ◽

2015 ◽

Vol 174 (4) ◽

pp. 9-12

Author(s):

A. A. Morozov ◽

R. R. Movsesyan ◽

V. G. Lyubomudrov

Keyword(s):

Blood Flow ◽

Surgical Treatment ◽

Pulmonary Blood Flow ◽

Interventricular Septum ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Definitive Repair ◽

One Step ◽

Pulmonary Arterial ◽

The Right

Pulmonary atresia with defect of interventricular septum and collateral pulmonary blood flow refers to complicated congenital malformation of the heart. Surgical treatment represents itself as very difficult task because of anatomical variability of this abnormality. The main problem of surgery is a definitive repair of the defect including correction of maldistributions of pulmonary arterial bed (unifocalization of pulmonary blood flow), reconstruction of outflow tract of the right ventricle and closing of interventricular septum defect. The performance of closing interventricular septum defect could be successful in patients with pulmonary atresia and collateral pulmonary blood flow in case of stepwise and one-step surgical treatment. The combination of maximal number of pulmonary segments and sufficient development of central pulmonary arteries facilitates to progress of assigned task. Patients with the level of pulmonary-arterial index more than 170 mm/m² and integrity of pulmonary arterial bed, centralization of pulmonary segments (minimum 15) could be considered as a candidate for definite repair of the defect.

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Pulmonary atresia with intact septum: Growth of the right ventricle and tricuspid valve following primary procedure

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(96)82282-0 ◽

1996 ◽

Vol 27 (2) ◽

pp. 343-344

Author(s):

P.E.F. Daubeney ◽

Z. Slavìk ◽

B.R. Keeton ◽

R.H. Anderson ◽

S.A. Webber

Keyword(s):

Right Ventricle ◽

Tricuspid Valve ◽

Pulmonary Atresia ◽

Primary Procedure ◽

The Right

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Effect of a regional increase in alveolar pressure on pulmonary blood flow

Journal of Applied Physiology ◽

10.1152/jappl.1992.73.4.1291 ◽

1992 ◽

Vol 73 (4) ◽

pp. 1291-1296 ◽

Cited By ~ 2

Author(s):

L. E. Olson ◽

R. L. Wardle

Keyword(s):

Blood Flow ◽

Pulmonary Blood Flow ◽

Left Lung ◽

Dry Weight ◽

Test Lung ◽

Test Segment ◽

Control Segment ◽

Lung Segment ◽

Lung Blood Flow ◽

The Right

We examined whether wedging a catheter (0.5 cm OD) into a subsegmental airway in dog (n = 6) or pig lungs (n = 5) and increasing pressure in the distal lung segment affected pulmonary blood flow. Dogs and pigs were anesthetized and studied in the prone position. Pulmonary blood flow was measured by injecting radiolabeled microspheres (15 microns diam) into the right atrium when airway pressure (Pao) was 0 cmH2O and pressure in the segment distal to the wedged catheter (Ps) was 0, 5, or 15 cmH2O and when Pao = Ps = 15 cmH2O. The lungs were excised, air-dried, and sectioned. Blood flow per gram dry weight normalized to cardiac output to the right or left lung, as appropriate, was calculated for the test segment, a control segment in the opposite lung corresponding anatomically to the test segment, the remainder of the lung containing the test segment (test lung), and the remainder of the lung containing the control segment (control lung). The presence of the catheter reduced blood flow in the test segment compared with that in the control segment and in the test lung. Blood flow was not affected by increasing pressure in the test segment. We conclude that, in studies designed to measure collateral ventilation in dog lungs, the presence of the wedged catheter is likely to have a greater effect on blood flow than the increase in pressure associated with measuring collateral airway resistance.

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