Prenatal diagnosis of sustained bradycardia with 1 : 1 atrioventricular conduction.

OBJECTIVES: The aims of this study were to elucidate the clinical course of fetal bradycardia with 1 : 1 atrioventricular conduction, and to discuss the optimal management of affected fetuses in the second and third trimesters of pregnancy. METHODS: The hospital records of five fetuses with the diagnosis of bradycardia (100 bpm) with 1 : 1 atrioventricular conduction between 1981 and 2000 in our institution were reviewed. Atrioventricular conduction was evaluated by simultaneous M-mode echocardiographic tracing of the atria and the ventricles. RESULTS: The gestational ages at referral ranged from 19 to 36 (median, 25) weeks, and fetal heart rates ranged from 60 to 80 (median, 80) bpm. Postnatal electrocardiography revealed sinus bradycardia in four (two of which were siblings) of the five cases, and junctional rhythm in the remaining case. Two fetuses with congenital heart defects (CHDs) were delivered by Cesarean section but died postnatally. The three fetuses without CHDs were delivered vaginally and have survived to date for 6, 8 and 15 years. CONCLUSIONS: Fetal bradycardia with 1 : 1 atrioventricular conduction caused by sustained sinus bradycardia or wandering pacemaker is an important type of fetal arrhythmia. Further investigations with a larger number of cases are required to determine the risk factors for predicting the outcome of affected fetuses.

Quantitation of the global right ventricular function in children with normal heart and congenital heart disease: a right ventricular myocardial performance index.

Although the assessment of right ventricular (RV) function is important in the clinical management of children with congenital heart disease, available imaging techniques have been limited because of the complex geometry of the right ventricle. A new Doppler index combining systolic and diastolic time intervals (the Tei index) has been reported to be useful for the assessment of global RV function in adults. However, normal values in children, age-related changes, and the clinical utility of the Tei index with regard to congenital heart disease have not been demonstrated. The purpose of this study was to prospectively assess RV function in children with normal heart and congenital heart disease using the Tei index. The subjects included 150 healthy children and 43 patients with congenital heart disease (35 patients with atrial septal defects and 8 patients who had had a Senning operation). The index was defined as the sum of isovolumetric contraction time and isovolumetric relaxation time divided by ejection time and was measured from conventional RV outflow and inflow Doppler velocity profiles. The Tei index was not affected by age in healthy children (0.24 +/- 0.04). There was a significant difference in index rating between patients who had had a Senning operation (0.58 +/- 0.09) and healthy children (p < 0.01), but there was no significant difference between children with atrial septal defect (0.25 +/- 0.13) and healthy children. The Tei index is a feasible approach to use when assessing global RV function in children with congenital heart disease.

Effect of endothelium removal on prostaglandin and nitric oxide function in pulmonary resistance arteries in the lamb.

We have recently shown that isolated pulmonary resistance arteries of the fetal lamb have prostaglandin (PG) I2 based and nitric oxide (NO) based relaxing mechanisms, which are activated by oxygen (at neonatal levels) and bradykinin. The present study was carried out to ascertain whether these mechanisms remain operational after removal of the endothelium. Endothelium-denuded vessels pre-equilibrated at a neonatal Po2 were not affected by indomethacin (2.8 microM), while they contracted weakly to NG-nitro-L-arginine methyl ester (L-NAME, 100 microM). However, the latter response did not reach significance and resembled that of intact vessels at fetal Po2. Bradykinin (0.1-100 nM) dose dependently (from 1-3 nM upwards) relaxed endothelium-denuded arteries that had been precontracted with a thromboxane (TX) A2 analog (ONO-11113, 0.1 microM) or excess potassium (5 mM Ca2+ in K(+)-Krebs) at a neonatal Po2. The response was the same under the two conditions, but it was smaller than that of intact arteries. Bradykinin relaxation of ONO-11113-contracted arteries was completely or nearly completely inhibited by indomethacin and L-NAME. We conclude that endothelium-denuded, pulmonary resistance arteries maintain PG (conceivably PGI2) mediated and NO-mediated relaxing mechanisms. These extra-endothelial mechanisms are activated by bradykinin but not by oxygen.

[Usefulness of open lung biopsy for corrective surgery on incomplete atrioventricular septal defect associated with severe pulmonary hypertension and right to left shunting].

In congenital heart defects, it is sometimes difficult to decide the operative indication in patients, who has severe pulmonary hypertension (PH) and right to left shunting, from clinical symptoms and cardiac catheterization. We here report a case of incomplete atrioventricular septal defect. The patient is 9 months old with the Down's syndrome. The cardiac catheterization showed severe PH and right to left shunting. Preoperative mean pulmonary artery pressure was 75 mmHg, pulmonary systemic pressure to systemic pressure ratio (PP/PS) was 1.17, pulmonary systemic flow to systemic flow ratio (QP/QS) was 0.79, pulmonary systemic resistance to systemic resistance ratio (RP/RS) was 1.54 and pulmonary vascular resistance (PVR) was 9.6 woodunits.m2. These data suggested that the irreversible pulmonary vascular disease was present. However the PVR decreased from 9.6 to 3.6 woodunits.m2 after inspiration of 100% oxygen. We therefore performed open lung biopsy for further evaluation of the pulmonary vascular bed. The pathological findings obtained at lung biopsy indicated that there was no irreversible pulmonary vascular disease. This case was diagnosed suitable for a corrective surgery. Total repair was performed and the subsequent clinical course was satisfactory. We thus suggest that the open lung biopsy is useful to decide the surgical indication when it is difficult to determine an operative indication from hemodynamic measurement alone.

Fetal arrhythmias: intrauterine diagnosis, treatment and prognosis.

Fetal echocardiography can provide useful information for the evaluation of fetal arrhythmias. Between 1980 and 1993, 44 fetuses with arrhythmias were diagnosed in utero at 12 and 40 weeks of gestation in Kurume University Hospital. Fetal bradycardia, tachycardia and ectopic beats were revealed in 17, seven and 20 fetuses, respectively, and their clinical features and prognosis were evaluated. In the 17 fetuses with bradycardia, eight were associated with congenital heart defect, and six of these developed to fetal hydrops. Of the 17 fetuses, four died in utero, one was terminated, and six died after birth. The other six cases survived. Three of these had a pacemaker implanted after birth. In the seven fetuses with tachycardia, transplacental anti-arrhythmic drugs were administered in five cases and conversion of the arrhythmia was achieved in four. None of the cases was associated with any congenital heart defect, and none died. Three infants had paroxysmal tachycardia postnatally. In the 20 fetuses with ectopic beats, arrhythmia was observed postnatally in 10, but all of these were resolved within 3 months after birth. Fetal bradycardias carried a poor prognosis in most cases and further studies are required to establish effective treatment. Some cases of fetal tachycardia developed recurrent tachycardia postnatally. Close follow-up of the newborn is therefore necessary.

Involvement of endothelin-1 in hypoxic pulmonary vasoconstriction in the lamb.

1. Using isolated pulmonary resistance vessels from mature fetal lamb and chronically instrumented lambs (8-17 days old), we have examined whether hypoxic pulmonary vasoconstriction is sustained by activation of a constrictor mechanism or suppression of a dilator mechanism. 2. Hypoxia contracted both arteries and veins in vitro, and the contraction was greater with the former. After removing the endothelium, arteries responded faster to hypoxia, but the magnitude of the response remained unchanged. 3. Hypoxic arteries, unlike normally oxygenated arteries, did not contract with either indomethacin (2.8 microM) or N omega-nitro-L-arginine methyl ester (L-NAME, 100 microM). The same vessels relaxed with sodium nitroprusside (SNP, 0.001-10 microM) but not with bradykinin (0.1-100 nM). 4. Endothelin-1 (ET-1, 0.01-10 nM) contracted isolated arteries and veins under normoxic and hypoxic conditions. In both vessels, the contraction was fast in onset and subsidence, and was inhibited by the ETA receptor antagonist BQ123 (1 microM). The ET-1 precursor, big ET-1 (100 nM), also contracted arteries and veins, but compared with ET-1 its action was slower in development. Big ET-1 contraction, unlike ET-1 contraction, was curtailed by the inhibitor of the ET-1-converting enzyme, phosphoramidon (50 microM). 5. ET-1 (0.1-10 nM) had no effect on isolated arteries precontracted with a thromboxane A2 (TXA2) analogue (ONO-11113) and treated with BQ123 (10 microM). Under the same conditions, ET-1 relaxed the veins. Accordingly, in the absence of BQ123 treatment, the selective ETB receptor agonist IRL-1620 (0.1-100 nM) relaxed the contracted veins but not the arteries. 6. BQ123 (10 microM) inhibited the constriction of isolated arteries and veins to hypoxia. Likewise, in the conscious lamb a bolus of BQ123 (0.4 mg kg-1, injected into the pulmonary artery) curtailed the rise in pulmonary vascular resistance (Rpa) brought about by alveolar hypoxia without changing significantly systemic vascular resistance (Rao). Under normoxia, Rpa was insignificantly affected by BQ123. 7. The results indicate that pulmonary resistance arteries are more susceptible to hypoxia than the veins, and that hypoxic vasoconstriction does not require an intact endothelium to occur. Hypoxic tone is ascribed primarily to intramural generation of ET-1, while removal of the tonic action of a relaxant may only have an accessory role in the response.

Intracranial malignant fibrous histiocytoma: characterization of GFAP-positive cells in the tumor.

A case of malignant fibrous histiocytoma (MFH) arising in the cerebellopontine angle of a 57-year-old woman was reported. The immunohistochemical and electron microscopic findings indicated distinct fibrohistiocytic nature and myofibroblastic differentiation of this peculiar tumor. Immunohistochemistry disclosed that the tumor cells expressed monocyte/macrophage markers including CD-68, MAC 387, and alpha-1-antichymotrypsin. In addition, the neoplasm contained scattered glial fibrillary acidic protein (GFAP)-positive cells which made small nests in some areas. These GFAP-positive cells had bundles of densely packed intermediate filaments and scanty organellae in the cytoplasm, as demonstrated by an immunostained-semithin and serial-ultrathin section method. By immunostaining of MIB-1 and GFAP, and silver nucleolar organizer region (AgNOR) impregnation on serial sections, the GFAP-positive cells were not labeled by MIB-1 and their AgNOR counts averaged 1.13/nucleus. Thus, these GFAP-positive cells seem to have lower proliferating activity than neoplastic astrocytes. It is concluded that they may be nonneoplastic astrocytic cells involved by MFH.

Endothelium-denuded pulmonary resistance arteries from the fetal lamb: preparation and response to vasoactive agents.

A method was developed for preparing endothelium-denuded resistance arteries (mean internal diameter, 176 microns) from the term fetal lung to be used for tests in vitro. The endothelium was removed mechanically by passing a sandpaper-polished cat whisker through the lumen. Ultrastructurally, the preparation had a well-preserved internal elastic lamina which was facing the lumen without any endothelium superimposed. Its response to contractile agents (excess potassium, a thromboxane A2 analogue, endothelin-1) tended to be greater compared to the intact artery. Conversely, the relaxation to acetylcholine was abolished in the endothelium-denuded artery and, in its place, a modest contraction ensued. Sodium nitroprusside relaxation, unlike acetylcholine relaxation, was affected insignificantly by endothelium denudation. This preparation, in combination with the preparation of the intact resistance artery, is a useful, new tool for studying local factors responsible for pulmonary hemodynamic control in utero and through the transitional period at birth.

[Actinomycotic granuloma of the optic chiasma: a case report].

A case of actinomycotic granuloma of the optic chiasma and the optic nerve is reported. A 53-year-old man was admitted to our hospital with headache and vomiting on September 25, 1989. General physical and neurological examination on admission revealed no remarkable findings. CT-scan demonstrated almost normal pictures. On the 17th hospital day, his temperature was 38 degrees C and white blood cell (WBC) count was 12000 cumm. And he presented right abducens palsy. MRI demonstrated no abnormal findings then. After antibiotic therapy of penicillin intravenously for 4 days, his temperature returned to normal and abducens palsy disappeared for two weeks. He was discharged 43 days after admission. But one year after discharge he developed progressive impairment of right visual acuity. He was admitted to our hospital again on January 19, 1991. His neurological examination revealed severe impairment of right visual acuity and bitemporal hemianopsia. WBC count was 5400 and CRP was negative and temperature was 36.2 degrees C. CT-scan and MRI demonstrated a suprasellar enhancing mass lesion. Serum prolactin was 38 ng/ml. A diagnosis of pituitary adenoma was considered and a right frontotemporal craniotomy was performed. At operation, abnormally swelled right optic nerve and chiasma were found and the swelled chiasma only was resected. Histological examination showed an abscess containing necrotic tissues and actinomycotic granules with radiating filaments. The patient was given penicillin intravenously for 37 days and orally for two months. At that time, MRI revealed no abnormal enhancing lesion, and his visual acuity improved gradually. Actinomycosis of the optic nerve and chiasma is very rare.(ABSTRACT TRUNCATED AT 250 WORDS)

Balloon valvuloplasty for congenital aortic valve stenosis in an infant and children.

Percutaneous balloon aortic valvuloplasty (BAV) was performed in 14 patients, including one critically ill infant with congenital valvular aortic stenosis (AS). BAV was effective in 13 patients (except the infant). The peak systolic pressure gradient between the left ventricle (LV) and the ascending aorta decreased from 76.6 +/- 21.6 to 29.5 +/- 15.3 mmHg (P less than 0.001). Follow-up cardiac catheterization was performed for eight patients between 1 and 3 years (1.6 +/- 1.1 years) after BAV. Restenosis was found in only one patient, and the efficacy of BAV continued significantly. Aortic regurgitation developed or increased in severity in 5 of 13 children immediately after BAV. Any other severe complication was not observed. Dilatation by BAV was not sufficient for the infant with critical AS, and acute myocardial infarction (AMI) in the lateral wall of the LV occurred during the BAV procedure. The infant died 3 days after the procedure due to AMI. It was concluded that the retrograde double balloon technique was superior to the retrograde single balloon technique. In two cases, the single balloon technique was ineffective because it was impossible to fix the balloon at the aortic annulus. However, the double balloon technique was effective in every patient. BAV is effective for AS in children, and an optional repeat trial may enable BAV to be the first choice for AS. Although BAV may be effective for neonates and infants with critical AS as an emergency treatment, much attention must be paid during the procedure.

[Arterial switch operation for transposition of the great arteries without use of prosthetic material].

For transposition of the great arteries (TGA), arterial switch operation (ASO) is theoretically preferable to atrial switch operation, since the left ventricle is established as the systemic ventricle and sinus node function is maintained. However, ASO is a delicate operation requiring the transfer of the coronary arteries. Use of prosthetic material for reconstruction of the neo-pulmonary artery causes postoperative supra-valvular pulmonary arterial stenosis. Five neonates and young infants with TGA underwent ASO without the use of prosthetic material by the technique reported by Pacifico et al. Three patients with simple TGA ranged in age from 10 to 27 days, and in weight from 2.9 to 3.9 kg. Two patients with TGA and ventricular septal defect ranged in age from 41 to 63 days, and in weight from 2.8 to 4.2 kg. There were no deaths, either early or late, and all patients are well 7 to 21 months after surgery. Catheterization and Doppler echocardiographic studies performed in all patients showed that pressure gradient between the right ventricle and pulmonary artery decreased significantly during mean follow-up period of 14 months. These results suggest excellent growth of the pulmonary artery after surgery.

Successful treatment of neonatal aneurysmal dilatation of the vein of Galen: the role of prenatal diagnosis and trans-arterial embolization.

We report a neonate with aneurysmal dilatation of the vein of Galen diagnosed prenatally by color Doppler sonography and MRI at 37 weeks' gestation. The child was treated by transarterial embolization of micro-coils 2 and 8 days after birth. The aneurysmal dilatation of the vein of Galen markedly decreased after embolization and the patient is developing normally at 2 years 5 months of age. Prenatal diagnosis and early intervention by transarterial embolization produced in a good outcome in this patient.

[Percutaneous balloon valvuloplasty for congenital aortic valve stenosis].

We performed percutaneous balloon aortic valvuloplasty for 10 patients with congenital aortic valve stenosis aged from 2 to 17 years and a 54-day-old infant with critical stenosis. The retrograde single balloon technique was used for 6 patients including the infant; the retrograde double balloon technique was used for 3 patients; and both techniques for 2 patients. The valvuloplasty was effective for 10 patients except for the infant in terms of the peak systolic pressure gradient between the left ventricle and aorta (from 80.6 +/- 21.9 to 29.4 +/- 17.0 mmHg). Follow-up cardiac catheterizations one year after valvuloplasty in 3 patients and 3 years after valvuloplasty in one patient disclosed no re-stenosis. Aortic regurgitation newly developed in one patient and advanced Sellers' classification I in 3 patients, however, all of them were asymptomatic and did not progress further. In the infant with critical stenosis, sufficient dilatation could not be achieved and acute myocardial infarction mainly at the lateral wall of the left ventricle developed during the valvuloplasty. He died 3 days after the valvuloplasty. The double balloon technique was found to be superior to the single balloon technique with the latter being ineffective in 2 cases, because the fixation of the balloon at the annulus was very difficult. Double balloon technique has low risk of vascular trauma and is applicable to a large sized annulus, because it enables blood supply between the 2 balloons during the inflation period.

[Cine magnetic resonance imaging for evaluating flow dynamics in congenital heart diseases with left-to-right shunts].

Cine magnetic resonance imaging (cine MRI) was used to evaluate the cardiac structures and blood flow in congenital heart diseases with left-to-right shunts. Fifteen children with left-to-right shunts which were confirmed by echocardiography or angiography were investigated in the present study. Five children each had atrial septal defect, ventricular septal defect, and complete endocardial cushion defect. Their ages ranged from 4 months to 13 years (mean 5.5 years). Prior to cine MRI, the ECG-gated cardiac imaging using multi-slice acquisition was performed in all the children to localize the optimal slice for cine MRI. To select the optimal imaging planes for various cardiac structures, we used axial, coronal, sagittal and four-chamber views. Cine MRI was demonstrated by a fast low 30 degree flip angle imaging technique, with a 15 msec echo time, a 30-40 msec pulse repetition time, and a 256 x 256 or 128 x 128 acquisition matrix. Abnormalities of cardiac structures were defined extremely well in all the children using ECG-gated cardiac imaging. In 14 of the 15 children (93%), cine MRI clearly detected a left-to-right shunt flow, which was visualized as a low signal intensity area compared to the surrounding blood flow. Noninvasively, cine MRI provides accurate images of the anatomy of the cardiac structures, makes functional assessments of the cardiac chambers and walls, and flow relationships. It has no limitations of imaging planes imposed bones and lung, and is not associated with technical difficulties as required with echocardiography which has small cardiac window.(ABSTRACT TRUNCATED AT 250 WORDS)

Changes in coagulation and fibrinolysis and effects of ticlopidine and cisternal drainage in the acute phase following aneurysm rupture.

In this study, changes in blood coagulation and fibrinolysis in the acute stage of subarachnoid hemorrhage (SAH) from ruptured intracranial aneurysms were investigated, and the effects of the platelet aggregation inhibitor ticlopidine and cisternal drainage were evaluated. All of the 53 patients underwent early surgery, and 27 patients received ticlopidine postoperatively. Cisternal drainage was implemented in 15 cases of severe SAH treated after late 1986. Of the hematologic factors studied, the activated partial thromboplastin time, fibrin and fibrinogen degradation products, fibrinogen, and platelet aggregation rate were found to be abnormal in the acute phase of SAH, and the "intravascular factor" scale obtained was also noted to be significantly (p less than 0.01) related to the outcome. Ticlopidine was consistently effective in reducing platelet aggregation but had little effect on other blood parameters. Cisternal drainage resulted in a significantly (p less than 0.01) lower incidence of symptomatic vasospasm and higher rate of good outcome. These results suggest that ticlopidine therapy plus cisternal drainage is highly beneficial in SAH, and that coagulation and fibrinolysis data are useful in determining the prognosis.

Tuberculous brain infection located in an old cerebral infarct: CT changes with successful conservative therapy.

A case of tuberculous brain infection following tuberculous meningitis in a 67-year-old man is presented. It was located in an old cerebral infarct associated with left internal carotid artery occlusion. CT demonstrated capsule enhancement in the left temporal area after iodinated contrast medium. Chemotherapy with INH, RFP and SM diminished the lesion and the capsule disappeared thirteen months later. It is suggested that a relatively long clinical history together with the appearance of a thick-walled abscess-like lesion on the CT scan is consistent with the diagnosis of tuberculous brain infection, perhaps an abscess.

[Studies of two cases of ruptured intracranial aneurysm with disseminated intravascular coagulation].

Two cases of ruptured intracranial aneurysm with severe DIC were reported. One case recovered due to early treatment. A 65-year-old man was admitted to our hospital in June, 1987 because he was suffering from sudden severe headache and nausea. On admission, CT-scan angiograms disclosed moderate subarachnoid hemorrhage (SAH) and probable ruptured anterior communicating aneurysm. However definitive diagnosis was not made at that time. On the 15th day after SAH, he had high fever and low blood arterial pressure. His laboratory findings were consistent with DIC, and therapy of FOY was then started with transfusion of fresh blood two days after. His laboratory findings and clinical status improved gradually and he underwent uneventful operation of the aneurysm on the 42nd day after SAH. Another case concerns a 71-year-old woman who was admitted to our hospital in June, 1988 because she had lost consciousness. On admission, CT-scan disclosed severe SAH and next day an operation was performed. On the 6th day after SAH, she showed clinical and laboratory evidence of severe DIC and died two days later. The association between DIC and ruptured intracranial aneurysms have been rarely mentioned in past reports. But the association might occur more frequently than we have supposed, and so more immediate and careful diagnosis and proper treatment for DIC should be given.

[Valvular regurgitation in patients with Kawasaki disease and in healthy children: a pulsed Doppler echocardiographic study].

Valvular lesions in the acute stage of Kawasaki disease were observed using pulsed Doppler echocardiography. The subjects consisted of 65 patients with Kawasaki disease (2 months-6 2/12 years) who had been followed from the acute stage. The age-matched 113 controls were selected from 661 healthy children (2 months-14 years of age). In the acute stage of Kawasaki disease, tricuspid regurgitation (TR) was detected in 31 (48%), pulmonary regurgitation (PR) in 22 (34%) and mitral regurgitation (MR) in 17 (26%). There was no aortic regurgitation (AR). In the convalescent stage, TR was found in 26 (40%), PR in 20 (31%), and MR in 11 (17%), but no AR was detected. The incidence of each valvular regurgitation between the acute and convalescent stages in patients with Kawasaki disease did not differ significantly. Furthermore, there was no significant difference in the incidence of valvular regurgitation between patients with Kawasaki disease and the normal controls. In nine patients, however, valvular regurgitation in the acute stage had disappeared by the convalescent stage, and two patients had developed a new pansystolic murmur in the acute stage. We estimated the incidence of pathologic valvular involvement in Kawasaki disease to be 11/65 (17%). The incidence of valvular involvement in patients with coronary artery aneurysms was significantly higher than that of patients without coronary artery aneurysms (p less than 0.01). It was concluded that mild and transient valvular regurgitation, which cannot be detected by auscultation, may occur in some patients in the acute stage of Kawasaki disease. These may be caused by acute inflammation of the valve related to coronary artery lesions. In view of the Doppler echocardiographic findings in normal controls, these regurgitations should be distinguished from "physiological" ones.

Hemodynamic evaluation and morphological changes of right ventricular out-flow tract and pulmonary valve after balloon pulmonary valvuloplasty.

Balloon pulmonary valvuloplasty (BPV) was attempted in 38 cases of congenital pulmonary valve stenosis. It was effective and was done without complication in 36 cases, however it was not effective in two cases of pulmonary valve dysplasia. The balloon used was 20-50% larger in diameter than the pulmonary valve annulus. In the seven cases in which the transvalvular pressure gradient was above 100 mmHg on cardiac catheterization, right ventriculography demonstrated that the functional obstruction of the right ventricular outflow tract increased immediately after BPV, however it subsequently improved at one year after the procedure. At between one and three months after BPV, two-dimensional echocardiography demonstrated that the morphological obstruction to the right ventricular outflow tract had diminished. In two cases, localized right ventricular septal hypertrophy with severe pulmonary valve stenosis was observed by two-dimensional echocardiography and right ventriculography, and persisted at one year. In all cases, two-dimensional echocardiography and angiography demonstrated doming pulmonary valves with valve stenosis, which was diminished by BPV. The pulmonary valve morphology was observed by two-dimensional echocardiography in three cases following BPV. In one case, partial relief of pulmonary valve obstruction was seen to be due to commissural splitting and in the other two, to tearing of cusp tissue. Inspection of the pulmonary valve at operation was made in one case who underwent elective surgery for repair of an atrial septal defect which was associated with pulmonary valve stenosis. It demonstrated partial relief of pulmonary valve stenosis by tearing of cusp tissue. In conclusion, BPV is as effective a treatment for congenital pulmonary valve stenosis as open valvulotomy. In our follow-up study, it has shown no apparent complications. The transient obstruction in the right ventricular outflow tract immediately after BPV improved within one month and improved further after three months and one year. However, in the more long-standing cases the localized right ventricular septal hypertrophy persisted and these cases will require, a long term follow-up.