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[A successful case report of one and one half ventricle repair for pure pulmonary stenosis in a 4-year-old girl].

A 4-year-old girl with pure pulmonary stenosis, hypoplastic right ventricle and atrial septal defect, underwent left modified Blalock-Taussig shunt at the age of 2 year. Her RVEDV was 62.7% of normal and TVD was 64.2% of normal at the age of 3 year. We observed development of right ventricle and performed simultaneously Glenn shunt and right ventricular outflow reconstruction (one and one half ventricle repair). Her general condition after operation became better. The size of tricuspid valve and right ventricle grew on Cardiac ultrasonography and catheterization examined after one year operation. In future, If the size of RV and TV might grow further, we should recommend her biventricular repair.

Child, Preschool↗

Pure infundibular pulmonary stenosis induced by radiation therapy--a case report.

A sixty-one-year-old woman developed acquired infundibular pulmonary stenosis eleven years after radiation therapy to the mediastinum. On catheterization, there was a 48 mmHg gradient between the right ventricle and the pulmonary artery. Postmortem examination revealed remarkable narrowing of infundibulum. Histologic examination strongly suggested that muscular hypertrophy of the right ventricular outflow tract had been induced by a high dose of radiation therapy.

Breast Neoplasms↗

Left ventricular ultrastructure in pulmonary stenosis and in tetralogy of Fallot.

Twelve patients underwent haemodynamic studies and myocardial biopsies: 7 with pulmonary stenosis (PS) and 5 with tetralogy of Fallot (TOF). Their ages ranged between 2 and 43 years. Right ventricular pressure was 128 +/- 43 mmHg in PS and 98 +/- 8 mmHg in TOF. Aortic blood oxygen saturation was 97.0% +/- 1.4% in PS, and 88.4% +/- 6.3% in TOF. Left ventricular (LV) weight was normal in TOF while it was increased in PS: 140.7 +/- 74.3 vs 74.0 +/- 8.7 g/m2 (P less than 0.001). Contractility was altered in both PS and TOF: ejection fractions were 56% +/- 7% vs 65% +/- 6% (P less than 0.001). Light microscopy showed abnormal transverse diameter of left ventricular myocytes in both PS and TOF: 18.6 micron +/- 4.0 micron vs 19.4 micron +/- 4.9 micron. The percentage of interstitium was normal: 29.6% +/- 3.9% vs 26.2 +/- 5.1%. Transmission electron microscopic examination revealed hypertrophic changes in all patients and degeneration in 7 of them. Hyperfunctional alterations of the myocytes were characterized by the increased number and reduced size of mitochondria, the enlarged Golgi complex, the increased number of ribosomes, the marked folding and convolutions of the nuclear membrane, the dilatation and tortuosity of T tubules. Myofibrillar lysis was the major degenerative change, which was also observed in the right ventricle (RV) of the same patients. No correlation was observed between these alterations and the patient ages, RV pressures, aortic blood oxygen saturations and ejection fractions. These findings led us to conclude that: (1) suprasystemic pressure overload of the RV induces macroscopic LV hypertrophy; (2) mild and suprasystemic pressure overload of the RV induces hyperfunctional changes in the LV; (3) myocardial degeneration is not related to hypertrophy nor to hypoxia, but is part of a more widespread cardiovascular fetopathy.

Adolescent↗

[Pulmonary artery aneurysm-like associated with pulmonary stenosis].

A 55-year old man had complained shortness of breathing and an abnormal shadow at left hilus region had been pointed out on the chest X-ray film. Right heart catheterization and right ventriculography revealed pulmonary valve stenosis with pulmonary artery aneurysm-like. Utilizing cardiopulmonary bypass, aneurysmorrhaphy and valvotomy was performed. Postoperative course was good and he was discharged on the 29th postoperative day. It is considered that mild post-stenotic dilatation of pulmonary trunk due to pulmonary valvular stenosis might develop into the aneurysmal change because of some congenital structural deficiency in the pulmonary arterial wall.

Aneurysm↗

Surgical management of large bronchial collateral arteries with pulmonary stenosis or atresia.

Single or multiple large bronchial collateral arteries may provide all or some of the pulmonary arterial blood flow in patients with proximal atresia of the pulmonary artery, and even in patients with only pulmonary stenosis. At the time of corrective surgery such arteries must be ligated in order to provide favorable operating conditions, to avoid cardiac overdistention during repair, and to prevent left-to-right intrapulmonary shunting postoperatively. Their ligation and control require precise preoperative definition of their number, origin, and course, and special intraoperative methods for their exposure. Associated hypoplasia of the pulmonary arteries may be severe enough to preclude corrective operation, but these hypoplastic arteries may enlarge in response to increase of blood flow through them resulting from a surgically created shunt. Experience with 14 surgically managed cases of this type forms the basis for the report.

Adolescent↗

Pulsed wave and color Doppler echocardiography and cardiac catheterization findings in bilateral pulmonary vein stenosis.

Pulmonary vein stenosis is a rare condition that usually is congenital in origin; it is almost invariably fatal in its bilateral and severe forms. It is often overlooked, however, during clinical examination, routine echocardiography, and even at cardiac catheterization. This report describes the pulsed Doppler, color flow echocardiography, and hemodynamic findings of bilateral pulmonary vein stenosis that accompany ventricular septal defect and atrial septal defect corrected by surgery.

Adult↗

Anaesthetic management of a parturient with pulmonary stenosis and aortic incompetence for Caesarean section.

Anaesthetic management of Caesarean section in a parturient with severe pulmonary stenosis and aortic regurgitation is described. The valvular sequelae resulted from previous unsuccessful surgical correction (Ross procedure) of congenital aortic stenosis. This case demonstrates the importance of multi-disciplinary assessment and careful anaesthetic planning, to avoid deterioration in perioperative cardiac performance in parturients with complex valvular disease.

Adult↗

[Double-outlet left ventricle with subaortic interventricular defect and pulmonary stenosis: surgical correction with cryopreserved aortic homograft].

Double outlet left ventricle with subaortic ventricular septal defect and pulmonary stenosis is a very rare congenital heart disease. We present a case of this cardiopathy which underwent correction consisting of ventricular septal defect closure and right ventricular outflow tract reconstruction using a cryopreserved aortic homograft, with a postsurgical follow-up time of 18 months.

Blood Vessel Prosthesis Implantation↗

Essential osteolysis associated with nephropathy, corneal opacity, and pulmonary stenosis.

We report on 5-year-old girl with essential osteolysis, nephropathy, corneal opacity, and valvular pulmonary stenosis. The patient was initially seen for evaluation of flexion contractures at wrists, elbows, and knees. Radiographic examination showed osteolytic changes primarily involving the hands and feet. She had persistent proteinuria; renal biopsy disclosed focal glomerulosclerosis in 1/3 of glomeruli. Electron microscopic study of skin fibroblast showed dilated and vacuolated rough endoplasmic reticulum. To our knowledge essential osteolysis associated with the aforementioned disorders has not been previously reported.

Child, Preschool↗

Subvalvular pulmonary stenosis, demyelination and myelodysplasia with monosomy 7.

We report a 23-year-old male with subvalvular pulmonary stenosis (PS) presenting with concurrent acute demyelinating neuropathy and myelodysplasia with monosomy 7. There was no stigmata of Noonan syndrome or neurofibromatosis, nor any family history of cardiac or hematologic disorders. There are 40 reported pediatric cases of Noonan syndrome with subvalvular PS that developed leukemia, over two-thirds with monosomy 7. There is also a genetic overlap between Noonan syndrome and neurofibromatosis, which is also closely associated with demyelination and leukemia. The importance of recognition of rare clinical syndromes that may predispose to leukemia is discussed.

Adult↗

Transaortic closure of ventricular septal defect in atrioventricular discordance with pulmonary stenosis or atresia. Results in five patients.

In five patients (aged 5 to 11 years) with atrioventricular discordance, pulmonary stenosis or atresia, and origin of the aorta from the right ventricle, transaortic closure of the ventricular septal defect was performed. Double-outlet right ventricle was confirmed in four patients. All patients underwent placement of an extracardiac valved conduit from the anatomic left ventricle to the pulmonary artery. Neither operative death nor postoperative complete heart block resulted. Postoperative follow-up periods were from 3 months to 4 years (average 35 months), and one patient who had no evidence of complete heart block died suddenly 2 years postoperatively. Aortic regurgitation developed in two patients from possible trauma during the operation: Regurgitation was mild (causing symptoms) in one patient who had had poor surgical exposure with a subpulmonic ventricular septal defect and a well-developed subaortic conus; it was trivial (asymptomatic) in the other patient. Transaortic ventricular septal defect closure, suturing a patch on the anatomic left ventricular side in patients with atrioventricular discordance and double-outlet right ventricle or similar anomalies, might help to avoid postoperative complete heart block if the aorta is large and the subaortic conus is not well developed.

Aorta↗

Unequal pressures in the central pulmonary arterial branches in patients with pulmonary stenosis. The influence of blood velocity and anatomy.

Significantly different pressures in the right (RPA) and left (LPA) pulmonary artery were observed at catheterization in patients with pulmonary valvar stenosis and no branch stenosis. The lower pressures in the RPA showed a "valley" during systole and were similar in contour and amplitude to the main pulmonary arterial (MPA) pressure; the LPA pressure, however, had a normal contour, and the peak systolic and mean pressures were higher than those in the MPA and RPA. Angiocardiograms, phonograms, and a simple analysis of fluid mechanics suggest that this pressure pattern is related to (1) the high-velocity jet in the MPA and (2) the anatomy of the central pulmonary arterial branches (bifurcation), the LPA originating more distally than the RPA. The high-velocity jet bypasses the origin of the RPA and breaks up in the distal MPA near the origin of the LPA. The kinetic energy is then reconverted into pressure, causing the higher LPA pressures. In patients with transposition of the great arteries and subvalvar pulmonary stenosis, the anatomy of the main pulmonary arterial bifurcation is different from normal, the RPA originating more distally than the LPA. The high-velocity jet may bypass the origin of the LPA and break up near the more distal origin of RPA, and the pressures in the RPA can be higher than those in the MPA and LPA.

Adolescent↗

The dimensions of the right ventricular outflow tract and pulmonary arteries in tetralogy of Fallot and pulmonary stenosis.

Studies were undertaken of the cineangiograms in 196 consecutive patients entering two institutions with tetralogy of Fallot and pulmonary stenosis, none of whom had previously undergone a surgical procedure. The median age of the patients at the time of the study was 5.9 months. The diameters of the right ventricular infundibulum, pulmonary trunk, and the entirety of the right and left pulmonary arteries were measured (in millimeters), corrected for magnification, and expressed in standard deviation units (Z-values). The median values of the cineangiographically determined diameters of the right ventricular infundibulum and pulmonary trunk were smaller than those of 95% of normal individuals. The median values throughout the right and left pulmonary arteries were within the range of normal. Those of the distal branches of both the right and left pulmonary arteries were similar to the mean values in normal individuals. However, great variability of the dimensions between individuals, and along the pathway in individuals, characterized patients with tetralogy of Fallot. Diffuse narrowing of the pathways both proximally and distally was uncommon. The relation between the diameters of the pulmonary "anulus" and of the distal pulmonary trunk and origin of the left pulmonary artery explained the difficulty of extending an enlarging patch into a wide area distally in some patients.

Cineangiography↗

Brock transventricular pulmonary valvotomy in patients with pulmonary stenosis: long-term results.

As no long-term results of a blind opening up of the pulmonary valve either by balloon valvoplasty or closed Brock valvotomy have been published, we examined the outcome of 12 patients with valvar pulmonary stenosis 17 +/- 5 (11-22) years after surgery. The Brock valvotomy had been carried out at a mean age of 3 +/- 2.8 (0.2-8.5) years. The mean pressure gradient across the right ventricular outflow tract had been 116 +/- 45 (75-97) mmHg at the catheter study or 106 +/- 43 (40-160) mmHg as measured intraoperatively. The right ventricular pressure after the Brock procedure was measured in the operating room in five patients as 46 +/- 15 (30-60) mmHg. Seven patients had been recatheterized at a mean age of 9.5 +/- 2 (7-12.7) years; at that time the gradient across the pulmonary valve had been 20 +/- 14 (10-37) mmHg. At a mean age of 21.7 +/- 3 (15-26) years these and five further patients were reexamined by echo Doppler. This time the pressure gradient across the pulmonary valve was 13 +/- 6 (7-20) mmHg. Moderate pulmonary incompetence was present in four and mild incompetence in eight patients; two had mild tricuspid insufficiency. All except one patient, who had suffered a cerebrovascular accident before surgery, were in NYHA functional class 1 and pursuing a profession. From these data we conclude that the blind opening-up of the pulmonary valve achieves excellent long-term palliation.

Blood Pressure↗

Surgical management of pulmonary atresia, and critical pulmonary stenosis with intact ventricular septum.

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum has included 20 patients with pulmonary atresia and 3 patients with critical pulmonary stenosis. Group 1 consisted of 7 infants who were treated by transarterial pulmonary valvotomy. Four of them were lost shortly after surgery. Group 2 included 11 neonates and one infant who were treated by systemic-pulmonary shunt with 2 operative death. Group 3 included 4 infants who underwent primary prosthetic enlargement of the right ventricular outflow tract. Two of the 4 were lost in the early postoperative period. Eight of 13 patients in groups 1 and 2 who survived the initial palliative procedures underwent additional operations to enlarge the right ventricular outflow tract, and 5 patients survived. However, adequate right ventricular and tricuspid valve growth was obtained in only patients in whom the prosthetic enlargement of the right ventricle was performed in early infancy. These findings strongly suggest the necessity to decompress the right ventricle completely, as early as possible. In conclusion, 1) transarterial pulmonary valvotomy seldom provided satisfactory decompression of the right ventricle, and operative risk was high. 2) systemic-pulmonary shunt to re-establish pulmonary blood flow improved neonatal survival, although this procedure may not provide a satisfactory long term palliation. 3) primary or secondary operative enlargement of the right ventricle in early infancy provided decompression of the right ventricle and accelerated substantial right ventricular growth. For patients without an infundibulum or for those in whom attempts to induce tricuspid valve growth had failed, a Fontan type procedure seemed to be the procedure of choice.

Female↗