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Surgical management of critical pulmonary stenosis in the neonate.

Experience with 36 consecutive neonates less than 1 month of age with critical pulmonary stenosis (PS) with intact ventricular septum was analyzed to define the role of pulmonary valvotomy and of a concomitant systemic-pulmonary shunt as well as the impact of prostaglandin E1 (PGE1) therapy in the management of this disorder. Operative procedures included pulmonary valvotomy as an isolated procedure (Group 1, N = 22), pulmonary valvotomy plus a systemic-pulmonary shunt (Group 2, N = 8), pulmonary valvotomy with PGE1 therapy (Group 3, N = 5), and one miscellaneous procedure. The hospital mortality (+/- 70% confidence limits [CL]) by treatment group was as follows: Group 1, 54% (CL, 41-67%) (12/22); Group 2, 25% (CL, 9-50%) (2/8); and Group 3, 0 (CL, 0-32%); Group 1 versus Group 2 plus Group 3 (p less than or equal to 0.05). Patients managed with a shunt or perioperative administration of PGE1 experienced a significant improvement in early survival. Late postoperative angiography demonstrated exemplary right ventricular growth in the majority of patients, although important residual abnormalities of the outflow tract necessitating operative repair were frequently present (5-year and 10-year actuarial freedom from reoperation, 73 +/- 10% and 42 +/- 16%, respectively [+/- standard error of the mean]). This review illustrates the limitations of pulmonary valvotomy as an isolated therapeutic method in neonates with critical PS. Provision of a systemic extracardiac source of pulmonary blood flow, accomplished by a systemic-pulmonary shunt or PGE1 infusion continued postoperatively, is the most important determinant of early survival in this disorder.

Alprostadil↗

Pulmonary stenosis caused by extrinsic compression of an aortic pseudoaneurysm of a composite aortic graft.

Pulmonic stenosis and stenosis of the right ventricular outflow tract related to extrinsic compression have been described in patients with tumors, in a patient with a pericardial cyst, and in patients with vascular abnormalities as an unruptured sinus of Valsalva aneurysm, a giant coronary artery pseudoaneurysm and an aortic arch aneurysm. Composite graft replacement of the ascending aorta and aortic valve with reimplantation of the coronary arteries has some inherent complications. Our case report describes a patient with a pericomposite graft aneurysm presenting as a stenosis of the pulmonary artery, detected by Doppler echocardiography.

Adult↗

Pulmonary vein stenosis.

Pulmonary vein stenosis (PVS) is a rare disorder. Accurate diagnosis often requires anatomical examination. We report four children with pulmonary vein stenosis. Autopsy showed bilateral lesions in two patients who were thought clinically to have unilateral disease. A diagnosis of PVS was made at autopsy in the third case. Intimal and medial fibromuscular proliferation was noted in extrapulmonary and intrapulmonary veins. Some of the fibromuscular proliferation were eccentric, resembling organized thrombi. In one case a focal organizing thrombus was found in a clinically unobstructed but anatomically narrowed veno-atrial junction. In another case injection of contrast medium into the stenotic pulmonary vein (PV) showed anastomosis between PV and bronchial vessels as well as small pulmonary arteries. Bilateral hypertensive arteriopathy was observed in unilateral and bilateral PVS. Our histological finding of intrapulmonary venous lesions in the lobes in which PVS was not detected clinically suggests that during surgical correction of unilateral PVS multiple biopsies of the opposite lung may help to evaluate possible bilateral disease. Our study also suggests that thrombosis in a stenotic pulmonary vein may further compromise the lumen and contribute to the progression of pulmonary vein obstruction. The possible pathogenesis of bilateral pulmonary hypertensive arteriopathy in unilateral PVS also is discussed.

Biopsy↗

Doppler ultrasound evaluation of valvar pulmonary stenosis from multiple transducer positions in children requiring pulmonary valvuloplasty.

The modified Bernoulli equation has been used to determine the peak pressure difference across stenotic pulmonary valves. The direction of the poststenotic jet may be eccentric in relation to the axis of the pulmonary artery. Consequently, the maximal velocity obtained from the parasternal transducer position may inaccurately estimate peak pressure difference. Thus, Doppler-derived estimates of pressure difference from the parasternal, subcostal, apical, and suprasternal notch transducer positions were compared with peak-to-peak pulmonary artery to right ventricle catheter withdrawal pressure differences in 24 patients admitted for pulmonary valvuloplasty. Suprasternal, subcostal or apical transducer positions produced higher maximal velocities than the parasternal transducer position in 12 of 24 patients when the studies were performed before cardiac catheterization and in 8 of 12 patients when performed during cardiac catheterization. The Doppler-derived estimates using the highest maximal velocity predicted catheterization pressure difference accurately when the measurements were not performed simultaneously (y = 1.05x - 3.3, r = 0.86, standard error of the estimate +/- 18.7 mm Hg) and the correlation was closer when the measurements were performed simultaneously (y = 1.09x - 2.7, r = 0.97, standard error of the estimate +/- 9.4 mm Hg). The transducer position that yielded the highest maximal velocity in an individual patient was the same before and after valvuloplasty. In all groups, the correlation with pressure at cardiac catheterization was improved by using the highest maximal velocity rather than the parasternal maximal velocity.

Cardiac Catheterization↗

Comparison of pulmonary venous flow velocities and left ventricular diastolic and ejection time in patients with moderate mitral and aortic stenosis. Pulmonary venous flow velocities in mitral and aortic stenosis.

BACKGROUND: Doppler pulmonary venous flow velocities (PVFV) pattern are useful parameters in assessing the left ventricular diastolic functions. Both mitral stenosis (MS) and aortic stenosis (AS) lead to diastolic dysfunction. We compared PVFV and left ventricular diastolic and ejection time (ET) in patients with moderate MS and AS. METHODS: Forty-three patients with moderate MS (group 1), 65 patients with moderate AS (group 2), and 33 healthy subjects as controls (group 3) were included in this study. After obtaining standard measurements echocardiographically, diastolic period (DP), ET, the ratio of the DP to the ET (DP/ET), isovolumetric contraction time (ICT), isovolumetric relaxation time (IRT), peak systolic flow velocity (PS), peak antegrade diastolic flow velocity (PD), peak reversal flow velocity at atrial contraction (PRA), the ratio of the peak systolic to the diastolic flow velocity (PS/PD), deceleration time of the antegrade diastolic flow (PDDT), and pressure half time of the peak antegrade diastolic flow velocity (PDPHT) were measured. Mitral valve area (MVA), aortic valve area (AVA), systolic pulmonary artery pressure (PAP), peak and mean gradients were calculated with standard formulas. RESULTS: In univariate analysis, MVA was correlated with PDPHT and PDDT (r = -0.41; p < 0.01, r = -0.36; p < 0.05, respectively), also it was correlated with DP/ET (r = -0.57; p < 0.001). Mitral peak and mean diastolic gradient were correlated with PS/PD (r = -0.43; p < 0.01, r = -0.36; p < 0.05, respectively) and DP/ET (r = 0.51; p < 0.01, r = 0.46; p < 0.01, respectively). AVA was only correlated with DP/ET (r = 0.38; p < 0.05). Aortic peak and mean systolic gradient were correlated with PS/PD (r = -0.29; p < 0.05, r = -0.27; p < 0.05, respectively) and DP/ET (r = -0.38; p < 0.01, r = -0.40; p < 0.01, respectively). In the same analysis, PAP in patients in group 1 and 2 was correlated with PS/PD (r = -0.42; p < 0.01 and r = -0.40; p < 0.01, respectively) and also it was correlated with PD (r = 0.37; p < 0.05 and r = 0.27; p < 0.05, respectively) in both groups. CONCLUSION: Moderate MS and AS similarly affect the PVFV, and PS/PD correlates with hemodynamics similarly both in MS and AS. Nevertheless, PDDT and PDPHT correlate with solely MVA. IRT higher in AS than MS, though DP/ET and ICT higher in MS than AS, and DP/ET relates with the severity of both MS and AS.

Adult↗

[A case report of isolated infundibular pulmonary stenosis with pouch of the infundibular chamber].

We experienced a surgical correction of isolated infundibular pulmonary stenosis (IIPS) of a 50-year-old male patient. The infundibular tissue was resected by the transpulmonary approach, and no trace of ventricular septal defect was found. There was a couple of pouch-like structure in the infundibular chamber of the right ventricle. The etiology of this structure was likely to be related turbulence as blood was ejected from the right ventricle to the infundibular chamber. Surgical correction of IIPS over 50 years old had been rarely reported. In addition, it was the first case report of IIPS with pouch of the infundibular chamber.

Cardiomyopathy, Hypertrophic↗

Double outlet right ventricle with L-position of the aorta, D-loop, subaortic VSD, and pulmonary stenosis.

We describe a case of double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis treated successfully with cardiopulmonary bypass. We consider the clinical history and angiocardiographic and surgical findings of this rare anomaly. We stress the difficulties of reconstruction of the outflow tract of the right ventricle, because of the anomalous pathway of the right coronary artery, the posterior situation of the pulmonary artery, and the abnormal anatomy present in the outflow tract of the right ventricle.

Angiocardiography↗

Interventional catheterization management of perioperative peripheral pulmonary stenosis: balloon angioplasty or endovascular stenting.

There is limited reported experience of catheterization therapy for peripheral pulmonary stenosis (PPS) at a surgical site in the early postoperative period. We reviewed the clinical course of patients undergoing interventional catheterization for PPS at a surgical site < 7 weeks after surgery. Successful dilation (SD) was defined as > 50% increase in predilation diameter. From 1984 to 2000, 17 patients had 19 proximal pulmonary arteries dilated 1 to 46 (median 8) days postoperatively. Median age and weight were 3.1 year and 12.7 kg. Three arteries were initially occluded. Seventeen arteries had initial BD with postintervention imaging available in 15; 8 arteries had SD. The arterial diameter increased from 3.9 +/- 2.6 to 5.5 +/- 2.8 mm (P < 0.001). Nine of these arteries had stents placed with diameter increasing to 8.7 +/- 3.7 mm (P < 0.001 compared with post-BD diameter). Stents increased the diameter in all arteries and made four of four failed BD successful. In the two most recent procedures, stents were placed without prior BD with diameter increasing from 1.3 to 9 mm and 8.2 to 14 mm. A stent was placed in 1 of 7 arteries prior to 1993 and in 10 of 12 arteries thereafter (P < 0.004). Three patients prior to 1995 had catheterization-related deaths secondary to vessel rupture after BD. BD produces SD in approximately one-half of the procedures but is associated with mortality. Stent placement increases vessel diameter substantially more than BD alone. Stents reduce the acute complication rate and avoid early reoperation in this patient group.

Adolescent↗

Supravalvular pulmonary stenosis after anatomic correction of transposition of the great arteries: causes and prevention.

Twenty-two patients who underwent anatomic correction of transposition of the great arteries at the arterial level at the age of 8 weeks to 14 years were reinvestigated 1-4.6 years (mean 2.0 years) after operation. A Dacron tube was used to bridge the gap between the proximal pulmonary route and the distal pulmonary artery in four patients and a tube made of homologous dura mater (2.5 cm in diameter) was used in 18. The nonvalved conduit was placed on the right side of the ascending aorta in nine patients and on the left side in 13. Supravalvular pulmonary stenosis with a gradient of 45-95 mm Hg was found in three patients, all of whom had had the conduit placed on the right side. There was normal growth of the pulmonary artery proximal and distal to the stenotic area. Operation for the relief of the stenosis with insertion of a pericardial patch across the stenosed area was performed in two patients. We conclude that supravalvular pulmonary stenosis after anatomic correction of transposition of the great arteries occurs only when the new pulmonary artery is placed on the right side of the ascending aorta and that the use of a large tube of dura mater appears to allow for rapid growth in early life.

Adolescent↗

[Physical activity and work capacity of patients in the late periods after the surgical correction of pulmonary stenosis].

From analysis of the data on the physical activity and working capacity of 162 patients with valvular stenosis of the pulmonary artery and tetraology of Fallot before and after correction of the anomaly, it was found that only 26.5% of patients have good physical resources before operation. In the late periods after effective correction of the defect in all grades of stenosis and even in secondary stenosis of the right conus arteriosus, reverse dynamics of functional disorders occurs and high physical activity is restored in more than 90% of patients.

Adolescent↗

Severe infundibular pulmonary stenosis and coronary artery stenosis with ventricular tachycardia 24 years after mediastinal irradiation.

A 28-year-old man developed severe infundibular pulmonary stenosis (PS), coronary artery stenosis with sustained ventricular tachycardia (VT) 24 years after mediastinal irradiation (total amount of 40 Gray) for non-Hodgkin's lymphoma. Repair of right ventricular outflow tract and coronary artery bypass graft procedure were performed. Infundibular PS was successfully relieved after operation and VT was also controlled by medication. Mediastinal irradiation often causes various cardiac complications after a latent period. Therefore, continuous careful observation is mandatory in patients with the history of mediastinal irradiation.

Adult↗

Successful balloon pulmonary valvuloplasty for congenital valvular pulmonary stenosis in an octogenarian.

While balloon valvuloplasty has been widely used for the treatment of congenital valvular pulmonary stenosis (PS) in children and adults, its use in elderly patients is less common. An 80-year-old woman with congenital valvular PS received valvuloplasty with double-balloon technique. Right ventricle systolic pressure and pulmonary valve systolic pressure gradient decreased from 95 to 44 mm Hg and from 75 to 35 mm Hg, respectively. Follow-up Doppler echocardiography 2 months later showed further decrease in the transvalvular systolic pressure gradient to 29 mm Hg. The patient had symptomatic relief, and no major complication was noted. Balloon pulmonary valvuloplasty can be an effective treatment for elderly patients with congenital valvular PS.

Aged↗