Infundibular Stenosis Research Articles

Management of Stones Associated with Intrarenal Stenosis: Infundibular Stenosis and Caliceal Diverticulum

To review our experience with retrograde intrarenal surgery (RIRS) for management of conditions associated with intrarenal stricture and present a treatment algorithm based on the series. RIRS was offered to all patients with symptomatic intrarenal stenosis regardless of location if stone burden was 2 cm or less. With a combined endourology and lithotripsy table, patients with stones between 2 and 3 cm were also offered RIRS using a combined approach of RIRS and shockwave lithotripsy (SWL). A total of 108 patients with symptomatic stones and caliceal diverticulum or infundibular stenosis were included in the data analysis. A standard technique was used in all cases. Failures or patients not suitable for RIRS were treated with either percutaneous nephrolithotomy (PCNL) or laparoscopic surgery. Successful identification and dilation/incision of the stenotic opening was accomplished in 94% of cases. Seventy-five percent of stones were managed with basketing and/or holmium laser ablation. In these patients, 90% were stone free (<2 mm stone fragments). For stones between 2 and 3 cm, the use of holmium laser in combination with SWL provided stone-free rates of 75%. Five percent of patients needed PCNL because of larger stone burden and posterior location. With the appropriate equipment, RIRS provides a valid treatment option for patients with intrarenal strictures. While upper pole and midrenal lesions are ideal, lower pole segments may be approached as well. A treatment algorithm based on the results provides a simplified approach for the minimally invasive management of intrarenal stenosis.

Extracorporeal lithotripsy endoscopically controlled by ureterorenoscopy (LECURS): a new concept for the treatment of kidney stones—first clinical experience using digital ureterorenoscopes

To evaluate the feasibility and usefulness of extracorporeal lithotripsy endoscopically controlled by simultaneous flexible ureterorenoscopy (LECURS) and combined with Holmium laser lithotripsy for kidney stones surgery. Retrograde flexible ureteroscopy allowed stone visualization and intrarenal relocation when possible. Extracorporeal schock wave lithotripsy (ESWL) was performed under direct vision allowing constant focal zone adjustment for optimal stone fragmentation. Holmium laser lithotripsy was used simultaneously when necessary. Post-operative assessment included ultrasound at day one and abdominal CT scan 3 weeks later to evaluate for residual fragments. Six patients with kidney stones were recruited including 3 with relative contraindication to ESWL. One patient had a stone beyond an infundibular stenosis and 2 had multiple stones in a horseshoe kidney with an uretero-pelvic junction (UPJ) stricture. A 100 % stone fragmentation rate was obtained. Post-operative ultrasound was normal in 5 patients and showed slight peri-renal infiltration in the other. One patient developed an obstructive pyelonephritis requiring antibiotics and changing a double J to a ureteral catheter. No other complications were noted. Three patients (50 %) were stone free after LECURS. Three patients underwent a second-look procedure for small (<3 mm) retained residual fragments. No damage was seen with the digital ureteroscopes, laser fibers or stone baskets. This initial experience with LECURS has shown to be feasible and safe. This opens the opportunity for further evaluation of this approach in order to improve outcomes of both approaches.

Magnetic resonance angiography vs. angiography in tetralogy of Fallot

: To determine whether gadolinium-enhanced three-dimensional magnetic resonance angiography can provide a noninvasive alternative to diagnostic catheterization for evaluation of pulmonary artery anatomy in tetralogy of Fallot. Thirty-five consecutive patients with tetralogy of Fallot, who attended the cardiology outpatient department between January 2008 and December 2009, were included in the study. There were 21 males and 14 females, with a mean age of 9 ± 4.15 years (range, 3-21 years). Thirty-two patients had tetralogy of Fallot with varying severities of valvular and infundibular stenosis. Three patients had tetralogy of Fallot with pulmonary atresia. All patients underwent both cardiac catheterization with X-ray angiography and 3-dimensional magnetic resonance angiography within one month. Measurements of right and left pulmonary arteries and aortopulmonary collaterals were equal by both methods. There was a good correlation between magnetic resonance angiography and catheterization measurements of branch pulmonary arteries. Gadolinium-enhanced three-dimensional magnetic resonance angiography can be used as a reliable noninvasive alternative to X-ray cineangiography for delineation of pulmonary arterial anatomy in sick infants and young children, obviating the need for catheterization.

Transventricular balloon dilation and stenting of the RVOT in small infants with tetralogy of fallot with pulmonary atresia

The management of small infants with tetralogy of Fallot (TOF) with pulmonary atresia (PA) and hypoplastic pulmonary arteries can be very challenging. In three small infants (weight range 2,200-3,600 g, pulmonary trunk 2.0-3.2 mm), initial palliation consisted of sternotomy, transventricular puncture of the right ventricular outflow tract and atretic pulmonary valve, followed by balloon dilation (n = 1) or stent deployment (n = 2) from the right ventricle into the pulmonary trunk (stent diameter 5-6 mm, length 16 mm). The procedure resulted in adequate palliation with good anterograde flow to the pulmonary arteries and near normal saturations in all three patients (>92%); there was no associated morbidity. Additional transvenous stenting was required in all patients because of progressive muscular infundibular stenosis after a median of 3 months. Two patients evolved to full repair at the age of 5 months and one patient with multiple hilar stenoses requires additional percutaneous procedures through the stented RV outflow tract. Transventricular balloon dilation and stenting of the RVOT through medial sternotomy as initial palliation strategy appears a safe and well tolerated alternative treatment in small infants with TOF with PA and a hypoplastic pulmonary trunk.

Isolated Left Ventricular Apical Hypoplasia with Infundibular Pulmonary and Aortic Stenosis: a Rare Combination

Isolated left ventricular (LV) apical hypoplasia is a rare congenital cardiac anomaly which is not accompanied by other cardiac abnormalities, with the exception of two cases. We report a case of a 33-year-old male patient with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. We review a literature focusing on the characteristic magnetic resonance features and combined cardiac abnormalities.

Anesthetic management of a patient with hypertrophic cardiomyopathy and patent ductus arteriosus

Anesthetic management of a patient with hypertrophic cardiomyopathy and patent ductus arteriosus

Abstract 18727: Right Ventricular Pressure Overload is More Important than Volume Overload in Producing Contralateral Ventricular Dysfunction: A Congenital Heart Disease Model

Intro: It is known that right ventricular (RV) contractile failure predisposes the left ventricle (LV) to failure. Animal models in an acute setting indicate this phenomenon is due predominantly to RV volume overload causing both changes in LV geometry and suboptimal LV filling. We investigated the relative importance of chronic RV pressure versus volume overload in producing LV dysfunction using d-transposition of the great vessels with an atrial switch (TGA, RV pressure overload) and repaired tetralogy of Fallot with severe pulmonary valve insufficiency (ToF, RV volume overload) as model systems. Methods: We performed a single center retrospective analysis of cardiac magnetic resonance imaging (MRI) data. All adult patients with TGA and ToF who had useful MRI data were screened. Included ToF patients had pulmonary insufficiency without residual pulmonary or infundibular stenosis. Included TGA patients had mild or less aortic or tricuspid regurgitation. We assessed MRI derived LV and RV volume measurements. Correlations were assessed using Pearson’s correlation coefficient. Differences between correlation coefficients were assessed using a Fischer r to z transformation. Results: 32 TGA and 46 ToF patients met enrollment criteria with 35 and 72 MRIs respectively having data adequate for analysis. The groups were similar in terms of sex (58 versus 51% male) and age (29.6 +/- 6.0 versus 31.0 +/- 9.7) respectively. Patients with TGA demonstrated a strong correlation between RV and LV systolic function based on ejection fraction (EF) r=0.75. They also demonstrated a strong correlation between RV end-diastolic volume indexed to body surface area (EDVi) and LV EF (r=-0.56). Although there was a trend toward increased RV EDVi in patients with ToF (119ml/m2 versus 101ml/m2 for TGA p=0.11), ToF patients had a weaker correlation between RV and LV EF (r=0.46; p=0.02 compared to TGA) and a very weak correlation between RV EDVi and LV EF (r=-0.21). Conclusions: The present data indicate that in the chronic setting, RV pressure rather than volume overload is of greater importance in producing LV dysfunction, implicating shared muscle fibers, neurohumoral milieu, or relative unloading of the LV as playing a greater role in this process.

Tips and pearls for “true” dextroposition of the aorta in tetralogy of Fallot

Infundibular stenosis may develop secondary to ventricular septal defect, and transannular patch plasty can affect mortality and morbidity rates. Therefore, dextroposition of the aorta has been investigated in order to eliminate discrepancies in the literature. Figures and illustrations from the selected references have been investigated and "actual dextroposition of the aorta" has been evaluated as far as the aorta is visible. A careful examination of these figures revealed the following tips and pearls for accurate diagnosis of dextroposition of the aorta: Aorta and ventricular septal defect should be adjacent for a "true" dextroposition of the aorta; the plane where the aorta exits from the ventricle should penetrate the plane of the ventricular septal defect towards the right ventricle; if the aorta and ventricular septal defect intersect at one edge, the aorta may seem to be dextroposed; new diagnostic modalities are necessary to evaluate the actual dextroposition status of the aorta for the proper planning of treatment.

LEOPARD syndrome

LEOPARD syndrome (LS) is a rare hereditary disorder, characterised mainly by skin, facial and cardiac abnormalities. We report on the case of a six-year-old Djiboutian with typical features of LS. Multiple cardiovascular problems are described, including pulmonary infundibular, valvular and supra-valvular stenosis. A favourable course was observed after successful cardiac surgery. This is the first reported case of LS from the horn of Africa.

Ureteropyeloscopic treatment of large, complex intrarenal and proximal ureteral calculi

Study Type - Therapy (case series) Level of Evidence 4 What's known on the subject? and What does the study add? Large upper tract urinary calculi, greater than 2 cm, have historically been treated with percutaneous nephrostolithotomy. In general, there has been a growing interest in employing retrograde, flexible ureteroscopy and laser lithotripsy in select patients who are either poor medical candidates for percutaneous lithotripsy or who may prefer a less invasive intervention. Properly selecting patients for this approach, designing specific treatments based on complex stone presentation and offering general information with regard to long-term outcomes and surgical risks have historically been based on results from small, multicentre series lacking uniformity of technique and long-term outcomes. Our initial multicentre experience employing ureteroscopic techniques to treat large upper urinary tract calculi was presented in 1998. This current work represented the largest single-centre experience, accrued prospectively over 10 years, where there was uniformity of technique and treatment algorithms. This study frames an argument for retrograde ureteroscopic lithotripsy not only in those who are at high risk for percutaneous nephrostolithotomy but in all who present with large, non-infected stone burdens. To define the safety and efficacy of retrograde ureteroscopic lithotripsy in treating large, non-infectious intrarenal and proximal ureteral stone burdens. Between 2000 and 2011, 145 patients with 164 large (2 cm or greater in diameter on standard imaging) non-infectious upper intrarenal and proximal ureteral calculi were chosen for retrograde ureteroscopic lithotripsy. Patients were treated with small diameter flexible fibre-optic ureteroscopes and holmium laser lithotripsy by a single surgeon. Second-look ureteroscopy was performed in patients with the largest calculi in whom there was a high index of suspicion of significant residual fragments. Stone clearance was defined as no fragments or a single fragment less than or equal to 4 mm in diameter on standard radiograph and sonography at 3-month follow-up. Our study included 103 male patients and 42 female patients with an average age of 55 years (range 16-86 years) and a mean stone diameter of 29 mm (range 20-70 mm) including 36 partial staghorn stone burdens (mean diameter 37 mm). Overall, 266 ureteroscopies were performed on 164 stone burdens (1.6 procedures per stone burden), clearing 143 stone burdens (87%). The highest clearance rates were observed for proximal ureteral (97%) and renal pelvic (94%) stones, while the lowest clearance rates were observed for lower pole (83%) and staghorn calculi (81%). Three patients required subsequent percutaneous therapy due to infectious material encountered at the time of ureteroscopy or inaccessible stone burdens secondary to infundibular stenosis. There were five minor postoperative complications, including four fevers and one patient with gross haematuria and clot retention, with no major intraoperative complications. In select patients, large, complex, metabolic upper urinary tract calculi can be treated safely and efficiently with retrograde ureteroscopic techniques. Staged, retrograde, flexible ureteroscopy is an alternative to percutaneous therapy with acceptable efficacy and low morbidity.

Stenting Right Ventricular Outflow in an Infant With Tetralogy of Fallot and Well-Developed Pulmonary Arteries

A right ventricular outflow tract stent was implanted in a 1-month-old (3.5 kg) baby boy with tetralogy of Fallot and worsening cyanosis to relieve infundibular and pulmonary valve stenosis in the setting of well-developed pulmonary artery branches. This management allowed a symmetric and optimal growth of the pulmonary artery branches, with right and left pulmonary arteries measuring 7-mm (z-score, +1.3 SD) and 7.3-mm (z-score, +1.7 SD), respectively, 3 months after stent implantation. No signs of pulmonary overcirculation developed, and complete surgical repair was performed at the age of 6 months.

2197 FLUORO-LESS URETEROSCOPY: MINIMIZING INTRAOPERATIVE RADIATION DURING URETEROSCOPIC PROCEDURES

You have accessJournal of UrologyImaging/Radiology: Uroradiology II1 Apr 20122197 FLUORO-LESS URETEROSCOPY: MINIMIZING INTRAOPERATIVE RADIATION DURING URETEROSCOPIC PROCEDURES Ryan Hsi and Jonathan Harper Ryan HsiRyan Hsi Seattle, WA More articles by this author and Jonathan HarperJonathan Harper Seattle, WA More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2012.02.2371AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Fluoroscopy usage during endoscopic procedures exposes the patient and operating room staff to ionizing radiation. Little is known regarding the average range of exposure during these cases. Mean fluoroscopy usage time during ureteroscopy reported from recent literature is between 15-183 seconds. The purpose of this study was to determine if a minimalist approach to fluoroscopy usage during ureteroscopy could reduce overall intraoperative fluoroscopy time. METHODS Over a 9 month period, fluoroscopy usage for all cases involving retrograde ureteroscopy for a single surgeon was prospectively recorded. A protocol was developed to access the ureter with the ureteroscope without fluoroscopy usage, and minimize radiation at every step (Figure 1). A retrospective chart review was then performed on patient factors and intraoperative events. Figure 1. Utilization of fluoroscopy during specific steps in ureteroscopy. Step Protocol Prior to procedure Surgeon controlled fluoroscopy pedal Scout film Unnecessary; C-arm positioned based on external visual cues Retrograde pyelogram Unnecessary unless difficult access to upper urinary tract Initial wire placement Place under tactile guidance or directly through ureteroscope Ureteroscopic access Place under tactile guidance to mid ureter over wire or drive directly into ureter Stent placement Single-tap pedal to visualize proximal stent curl, visualize distal curl cystoscopically RESULTS Fluoroscopy usage during ureteroscopic cases was utilized on 109 renal units in 73 consecutive patients. The indications for ureteroscopy included the treatment of stones (84%), diagnosis and/or biopsy (15%), and laser incision of infundibular stenosis (1%). Of the 102 renal units with fluoroscopic usage data, total mean and median fluoroscopy time was 2.8 and 1.0 seconds (0-31 seconds), respectively. Excluding fluoroscopy usage to confirm ureteral stent placement, 72% of all cases did not require any fluoroscopy time, and 82% required 2 seconds or less. For the last 44 consecutive renal units, total mean and median radiation dose was estimated at 0.5 mGy and 0.4 mGy (0.0-2.3 mGy), respectively. The most common reasons for utilization of fluoroscopy during ureteroscopy included aberrant anatomy, severely impacted ureteral stone, or difficult navigation due to a tight ureter. There were no intraoperative complications. CONCLUSIONS The reduced fluoroscopy protocol resulted in minimal fluoroscopy time and radiation exposure, significantly lower than reported in the literature. Fluoro-less ureteroscopy is safe and feasible in the majority of ureteroscopic cases and lessens exposure to patients and staff. © 2012 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 187Issue 4SApril 2012Page: e886 Advertisement Copyright & Permissions© 2012 by American Urological Association Education and Research, Inc.MetricsAuthor Information Ryan Hsi Seattle, WA More articles by this author Jonathan Harper Seattle, WA More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Surgical correction of tetralogy of Fallot in a 61-year-old patient

Tetralogy of Fallot (TOF) is a congenital heart disease that is usually diagnosed and treated during infancy. Only 3% of such patients reach the age of 40 without surgery. We describe a 61-year-old woman with uncorrected TOF that was successfully treated by radical surgery. The patient presented with exertional dyspnea, insomnia, and malaise. Echocardiography and cardiac catheterization indicated a dilated, severely hypertrophic right ventricle, ventricular septal defect, an overriding aorta, and infundibular stenosis in the right ventricular outflow tract. All symptoms disappeared after full surgical correction, which remains the preferred treatment for adult TOF because it confers long-term survival and an improved quality of life.

Long term follow-up of 799 children with isolated ventricular septal defects

We evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD). The study included 799 patients (368 girls, 431 boys; mean age at diagnosis 24.3±37.4 months; median 6 months) who were monitored by the pediatric cardiology department for VSD. The mean follow-up period was 32.8±30.3 months (median 20 months). The VSDs were classified as perimembranous (n=610, 76.4%), muscular (n=171, 21.4%), doubly committed subarterial (n=10, 1.3%), and multiple (n=8, 1%). Spontaneous closure rates were 42.7%, 13.1%, and 25% in muscular, perimembranous, and multiple VSDs, respectively, which corresponded to a mean age of 18.6±19.9 months (median 12 months) in muscular and 30.2±33.7 months (median 14.5 months) in perimembranous VSDs. Before 2 years of age, 78.1% of muscular and 58.6% of perimembranous VSDs underwent spontaneous closure. Of 256 defects (32%) that required surgical closure, 91.4% were of perimembranous location. The mean age at surgery was 38.8±49.1 months (median 11 months) for muscular, and 43.7±40.9 months (median 24 months) for perimembranous defects. During the follow-up period, the following complications were noted: aortic valve prolapse (0.7%), aortic regurgitation (0.6%), left ventricle-to-right atrium shunt (2.6%), subaortic ridge (3.7%), and infundibular stenosis (1.2%). Aortic regurgitation developed in eight patients (3.7%) after surgical closure. Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients' age, defect type, and complications encountered in the follow-up period.

Primary Infundibular Stenosis and Pedigree Analysis in Three Golden Retriever Littermates

Three eight-week-old Golden Retriever puppy littermates were evaluated because of left basilar systolic murmurs and were diagnosed with primary infundibular stenosis. Pedigree analysis in this line was also performed to identify a mode of inheritance. All dogs were asymptomatic at the time of diagnosis; two of the three had congenital lesions in addition to primary infundibular stenosis. Two additional affected dogs were identified in the line, and pedigree analysis suggested an autosomal recessive mode of inheritance. Another, unrelated golden retriever was also identified with isolated infundibular stenosis in the record database. Primary infundibular stenosis should be considered in the differential diagnoses for golden retriever dogs with a left basilar systolic murmur, and is often associated with complex congenital cardiac disease. Primary infundibular stenosis may worsen in severity with time, and in this line of dogs an autosomal recessive pattern of inheritance is likely.

A case of single atrium and single ventricle physiology with bilateral cleft lip and palate for lip repair surgery

Bilateral cleft lip and palate may occasionally be associated with complex congenital cyanotic heart disease. An infant with common atrium and single ventricle with infundibular pulmonary stenosis (Blalock-Taussig shunt done recently) presented for lip repair surgery. Balanced general anesthesia was administered using sevoflurane along with a regional nerve block to maintain optimal pulmonary and systemic vascular resistance.

Left ventricular apical ballooning syndrome in a patient with infundibular stenosis of the right ventricle: A case report

A 73-year-old female patient with a past history of right ventricular infundibular stenosis was admitted to our intensive care unit because of right ventricular dysfunction. On the fifth day of hospitalization, she suddenly experienced dyspnea without chest pain despite the improvement of her condition by initial medical treatment. Although electrocardiography revealed no ST-segment elevation, echocardiography and myocardial perfusion using 99mTc-MIBI revealed new development of severe symmetrical akinesia and reduced perfusion of the left ventricular (LV) apex and mid-ventricle. LV apical ballooning syndrome was diagnosed based on the minimal elevation of cardiac enzymes (peak cardiac troponin I 0.18ng/ml) despite the presence of large regions of focal myocardial damage in the myocardium and the absence of positive ECG diagnosis and urgent coronary angiography. Previous coronary angiography revealed normal coronary arteries and the left anterior descending artery without full irrigation around the apex making apical ballooning. On the 12th day of hospitalization, despite the use of positive inotropic treatment, it was impossible to maintain hemodynamic stability, and the patient died prior to the functional recovery of the left ventricle.

Accessory mitral valve tissue: an unusual cause of congenital mitral stenosis

Accessory mitral valve tissue (AMVT) is a rare congenital anomaly of endocardial cushion. Usually, it arises from the anterior mitral leaflet and causes the left ventricular outflow tract obstruction. We report here the first and a rarest presentation of the AMVT in a 19-year old female patient diagnosed to have double outlet right ventricle, ventricular septal defect (VSD), infundibular stenosis and congenital mitral stenosis (MS). She presented with a history of shortness of breath and chest pain over 3 years. Diagnosis was made by chest X-ray, transthoracic and transoesophageal echocardiography, which was confirmed by cardiac catheterization, and angiography. A successful closure of the VSD with excision of the right ventricular bundle and excision of the AMVT was done. Post-operative course was uneventful and an echocardiogram before the discharge showed no residual shunt, no right ventricular outflow gradient or mitral regurgitation, and the gradient across the mitral valve was 5/3 mmHg. We emphasize that, although AMVT commonly produces left ventricular outflow tract obstruction, it should be considered a rare but an important cause of congenital MS.

Unilateral Multicystic Dysplastic Kidney With Progressive Infundibular Stenosis in the Contralateral Kidney: Experience at 1 Center and Review of Literature

We analyzed the association between unilateral multicystic dysplastic kidney and subsequent contralateral infundibular stenosis, which can result in progressive calyceal dilatation, and has been linked to global hyperfiltration injury and renal impairment. During the last 10 years 200 children presented with unilateral multicystic dysplastic kidney. Of these children 5 subsequently exhibited contralateral infundibular stenosis. We reviewed the published data on multicystic dysplastic kidney as well as infundibular stenosis to examine this association further. Three patients underwent open surgical exploration since rapid progression with associated parenchymal thinning was detected. Literature review failed to identify any discussion of infundibular stenosis in studies focusing on long-term followup of children with a unilateral multicystic dysplastic kidney. Several case reports and case series discuss this condition in association with other collecting system anomalies. However, these anomalies are hypothesized to be part of a disease spectrum resulting from aberrant formation of the collecting system. Bilateral involvement has been reported in fewer than 10 cases. Our cases represent a part of the spectrum of pyelocalyceal dysgenesis that can have bilateral involvement of varying degrees. Of particular concern was the delayed presentation in some of our patients and the progressive nature of the lesions. Although exceedingly rare, we wish to highlight the association of multicystic dysplastic kidney and progressive infundibular stenosis of the contralateral kidney and renal impairment.

Serum and pulmonary vascular endothelial growth factor/receptors and haemodynamic measurements in cyanotic congenital heart disease with decreased pulmonary blood flow

Tetralogy of Fallot is the most common cyanotic congenital heart disease with decreased pulmonary blood flow. Right-to-left shunt and infundibular pulmonary stenosis in this disease lead to a decrease in arterial O(2) saturation. Hypoxia is a strong stimulus for angiogenesis; however, the reason for insufficiency in the pulmonary vascular growth in patients despite chronic arterial hypoxia is still not known. This study was planned considering that the impairment in vascular endothelial growth factor-receptor relationship or the vascular endothelial growth factor-receptor deficiency in the pulmonary vascular bed during development may cause insufficiency of pulmonary vascular growth. A total of 24 patients were grouped as cyanotic - including 13 patients with tetralogy of Fallot - and acyanotic - including 11 patients with left-to-right shunt lesions. During cardiac catheterisation, vascular endothelial growth factor measurements were performed; and oxygen saturations, pressures, and haemoglobin levels were measured. Perioperative lung biopsy for vascular endothelial growth factor receptors was performed in the cyanotic group. Vascular endothelial growth factor of the aorta was higher in the acyanotic group. There was a significant negative correlation between vascular endothelial growth factor levels and aortic O(2) saturation in the cyanotic group (p < 0.05). Vascular endothelial growth factor tissue staining was negative in 11 out of 13 (84.6%) patients. KDR/Flk-1 receptor was positive in four out of 13 (30.7%) patients; Flt-1 receptor was positive in six out of 13 (46.1%) patients. Vascular endothelial growth factor values were found to be lower than those of the acyanotic patients in this study. Low serum vascular endothelial growth factor levels of the cyanotic group, in spite of the hypoxia, demonstrated the importance of studying vascular endothelial growth factor tissue levels and vascular endothelial growth factor receptors in these patients.