Evaluating TIMP-2 and IGFBP-7 as a predictive tool for kidney injury in ureteropelvic junction obstruction

ABSTRACT A major challenge in the management of ureteropelvic junction obstruction (UPJO) is the selection of patients who would benefit from surgical treatment. Tissue inhibitor metalloproteinase-2 (TIMP-2) and insulin-like growth factor-binding protein 7 (IGFBP7) indicate renal cell stress and are associated with cell cycle arrest. The [TIMP-2] [IGFBP7] ratio (Nephrocheck®) has been recently applied in patients in intensive care units patients to predict the development of acute kidney injury. In this study, we evaluated the performance of these biomarkers performance to distinguishing obstructive hydronephrosis (HN) from non-obstructive HN. Materials and Methods: Consecutive patients with UPJO were enrolled in this study. Urinary [TIMP-2] [IGFBP7] and clinical characteristics (hydronephrosis grade, differential renal function, and drainage half-time) were measured in the following groups: 26 children with obstructive HN at initial diagnosis (group 1A) and after six months of dismembered pyeloplasty (group 1B); 22 children with non-obstructive HN (group 2), and 26 children without any urinary tract condition, as the control group (group 3). Results: Comparing the initial samples, [TIMP-2] [IGFBP7] had higher levels in the HN groups and lower levels in the control group; however, no difference was observed between the HN groups (obstructive vs. non-obstructive). After six months of follow-up, patients who underwent dismembered pyeloplasty showed stability in the urinary concentration of [TIMP-2] [IGFBP7]. All patients with [TIMP-2] [IGFBP7] higher than 1.0 (ng/mL)2/1000 had diffuse cortical atrophy on ultrasonography. Conclusions: We showed that urinary levels of urinary [TIMP-2] [IGFBP7] are higher in children with HN than controls. Nephrocheck® is not reliable in predicting the need for surgical intervention for pediatric patients with UPJO.

Evaluating TIMP-2 and IGFBP-7 as a predictive tool for kidney injury in ureteropelvic junction obstruction.

A major challenge in the management of ureteropelvic junction obstruction (UPJO) is the selection of patients who would benefit from surgical treatment. Tissue inhibitor metalloproteinase-2 (TIMP-2) and insulin-like growth factor-binding protein 7 (IGFBP7) indicate renal cell stress and are associated with cell cycle arrest. The [TIMP-2] [IGFBP7] ratio (Nephrocheck®) has been recently applied in patients in intensive care units patients to predict the development of acute kidney injury. In this study, we evaluated the performance of these biomarkers performance to distinguishing obstructive hydronephrosis (HN) from non-obstructive HN. MATERIALS AND METHODS: Consecutive patients with UPJO were enrolled in this study. Urinary [TIMP-2] [IGFBP7] and clinical characteristics (hydronephrosis grade, differential renal function, and drainage half-time) were measured in the following groups: 26 children with obstructive HN at initial diagnosis (group 1A) and after six months of dismembered pyeloplasty (group 1B); 22 children with non-obstructive HN (group 2), and 26 children without any urinary tract condition, as the control group (group 3). RESULTS: Comparing the initial samples, [TIMP-2] [IGFBP7] had higher levels in the HN groups and lower levels in the control group; however, no difference was observed between the HN groups (obstructive vs. non-obstructive). After six months of follow-up, patients who underwent dismembered pyeloplasty showed stability in the urinary concentration of [TIMP-2] [IGFBP7]. All patients with [TIMP-2] [IGFBP7] higher than 1.0 (ng/mL)2/1000 had diffuse cortical atrophy on ultrasonography. CONCLUSIONS: We showed that urinary levels of urinary [TIMP-2] [IGFBP7] are higher in children with HN than controls. Nephrocheck® is not reliable in predicting the need for surgical intervention for pediatric patients with UPJO.

Recent advances in pediatric bladder malignancies.

Urothelial pediatric neoplasms are relatively rare. Papillary urothelial neoplasms of low malignant potential (PUNLMPs) and rhabdomyosarcoma (RMS) are the most common bladder malignancies in the pediatric population. Clinical presentation encompasses macroscopic hematuria or lower urinary tract symptoms (or both) or is detected incidentally at imaging. Tumors arising from the bladder can originate from any of its four histological layers (urothelium, lamina propria, detrusor, and adventitia) and are divided into tumors that have an epithelial origin (arising from the urothelium) and those that have a non-epithelial origin (mesenchymal neoplasms). RMS is the most common malignant tumor of the urinary bladder in children younger than 10 years. Deriving from the embryonic mesenchymal cell, the histopathologic subtypes of RMS are embryonal RMS (>90%) and alveolar histology (<10%). Pre-treatment imaging should be carried out by computed tomography (CT) or at present is more likely with magnetic resonance imaging of the pelvis. Chest CT and bone scintigraphy are used to screen for metastases. In selected cases, a positron emission tomography scan may be recommended to evaluate suspicious lesions. The current prognostic classification considers age, histologic subtype, tumor site, size, and extent (nodal or distant metastases). Staging is based on pre-operative findings, group is based on intra-operative findings and pathology, and risk stratification is derived from both stage and group data. Pre-operative chemotherapy is the most common first-line intervention for bladder/prostate RMS, before surgery or radiation therapy. Collaborative groups such as the Soft Tissue Sarcoma Committee of the Children's Oncology Group and the European Pediatric Soft Tissue Sarcoma Study Group endorse this therapy. PUNLMPs are generally solitary, small (1-2 cm), non-invasive lesions that do not metastasize. Therapy is usually limited to a transurethral resection of the bladder tumor. About 35% are recurrent and around 10% of them increase in size if they are not treated.

EMG biofeedback or parasacral transcutaneous electrical nerve stimulation in children with lower urinary tract dysfunction: A prospective and randomized trial.

AIMS: Evaluate the efficacy of biofeedback and parassacral electric nerve stimulation (TENS) for the treatment of children with lower urinary tract (LUT) dysfunction. METHODS: A prospective, randomized study was approved by our Hospital Ethics Committee. We enrolled 64 children, 43 girls and 21 boys, average age of 9.39 years. The initial evaluation consisted of history, physical examination, urine analyses, voiding diary, uroflow, and ultrasound. Dysfunction voiding symptom score (DVSS) questionnaires were applied pre- and post-treatment. The children were divided into two treatment groups independent of the predominant type of voiding dysfunction (dysfunctional or overactive bladder): biofeedback group and TENS group. The criteria for assessing the effectiveness of the techniques was the resolution of daytime and nighttime symptoms including urinary leakage, improvements in voiding diary, DVSS, and changes in uroflow. After 6 months, the children were reassessed with the same work-up of baseline. RESULTS: Regarding daytime symptoms, results for complete response were similar between the two groups (P = 0.483); 54.9% of children treated by the biofeedback group and 60.6% in the TENS group. The same have been observed in the nighttime incontinence with complete resolutions in 29.6% and 25%, respectively ( P = 0.461). Analyzing the voiding diary, uroflow and DVSS questionnaires both groups had significant improvement ( P = 0.001) after treatment. The biofeedback group required fewer sessions than TENS group, 10.9 and 18.1, respectively ( P < 0,001). CONCLUSIONS: Both biofeedback and the TENS are equally effective for treating non-neurogenic voiding dysfunction. Biofeedback seems to require a lower number of sessions to obtain similar results of the TENS.

Single-stage Abdominoplasty Using Groin Flaps Without Osteotomies: Management of Exstrophy-epispadias Complex.

BACKGROUND AND OBJECTIVE: The optimal treatment for children born with exstrophy-epispadia complex is still a matter of debate.1,2,3 We demonstrate the Single-Stage Abdominoplasty using Groin Flap technique to close the abdominal wall of children with classic bladder exstrophy (CBE) without osteotomy neither radical soft tissue mobilization. Advantages over current techniques are less risk of penile tissue loss and avoidance of osteotomies. MATERIAL AND METHODS: Abdominal wall repair consists in using the hypogastric skin, rectus, and obliquus externus abdominalis muscle fascial flaps. These groin flaps are rotated medially resulting in a very strong abdominal wall support. Groin flaps are made of rectus anterior fascia rotated medially, flipped over, and sutured with Prolene sutures to close the defect. By rotating the fascial flaps medially, complete reinforcement of the abdominal wall to the level of the pubic bone is achieved. This permits abdominal closure maintenance without tension. RESULTS: Groin flap was applied to 128 patients with CBE referenced from all over the country. Most of these patients returned to their home areas making difficult their follow up. However, we have 44 cases that have regular clinical visits. Mean follow-up was 10.3 ± 4.5 years (2 years 8 months-16 years). Successful closure was achieved in 43 patients (97.7%) as a single procedure; one patient had a complete wound dehiscence and needed another reconstruction (2.2%). Four patients (9.1%) presented abdominal hernias that needed surgical management. When continence is evaluated, we present similar literature rates (60%).4 CONCLUSION: Abdominal reconstruction using Groin flaps has advantages over the traditional approaches to CBE. It reduces the surgical steps and facilitates the closure of the abdominal wall without the need of osteotomies and consequent immobilization during the postoperative period. It is feasible at any age and can be also very useful as a salvage technique even after previous failed procedures. Finally, it minimizes the number of surgeries.

Transperitoneal laparoscopic pyeloplasty in children: does upper urinary tract anomalies affect surgical outcomes?

ABSTRACT Objective To assess the feasibility and outcomes of laparoscopic pyeloplasty in children with complex ureteropelvic junction obstruction (UPJO) and compare to children with iso-lated UPJO without associated urinary tract abnormalities. Material and Methods Medical records of 82 consecutive children submitted to transperi-toneal laparoscopic pyeloplasty in a 12-year period were reviewed. Eleven cases were con-sidered complex, consisting of atypical anatomy including horseshoe kidneys in 6 patients, pelvic kidneys in 3 patients, and a duplex collecting system in 2 patients. Patients were di-vided into 2 groups: normal anatomy (group 1) and complex cases (group 2). Demographics, perioperative data, outcomes and complications were recorded and analyzed. Results Mean age was 8.9 years (0.5-17.9) for group 1 and 5.9 years (0.5-17.2) for group 2, p=0.08. The median operative time was 200 minutes (180-230) for group 1 and 203 minutes (120-300) for group 2, p=0.15. Major complications (Clavien ≥3) were 4 (5.6%) in group 1 and 1 (6.3%) in group 2, p=0.52. No deaths or early postoperative complications such as: urinoma or urinary leakage or bleeding, occurred. The success rate for radiologic improvement and flank pain improvement was comparable between the two groups. Re-garding hydronephrosis, significant improvement was present in 62 patients (93.4%) of group 1 and 10 cases (90.9%) of group 2, p=0.99. The median hospital stay was 4 days (IQR 3-4) for group 1 and 4.8 days (IQR 3-6) for group 2, p=0.27. Conclusions Transperitoneal laparoscopic pyeloplasty is feasible and effective for the management of UPJO associated with renal or urinary tract anomalies.

Transperitoneal laparoscopic pyeloplasty in children: does upper urinary tract anomalies affect surgical outcomes?

OBJECTIVE: To assess the feasibility and outcomes of laparoscopic pyeloplasty in children with complex ureteropelvic junction obstruction (UPJO) and compare to children with iso-lated UPJO without associated urinary tract abnormalities. MATERIAL AND METHODS: Medical records of 82 consecutive children submitted to transperitoneal laparoscopic pyeloplasty in a 12-year period were reviewed. Eleven cases were con-sidered complex, consisting of atypical anatomy including horseshoe kidneys in 6 patients, pelvic kidneys in 3 patients, and a duplex collecting system in 2 patients. Patients were di-vided into 2 groups: normal anatomy (group 1) and complex cases (group 2). Demographics, perioperative data, outcomes and complications were recorded and analyzed. RESULTS: Mean age was 8.9 years (0.5-17.9) for group 1 and 5.9 years (0.5-17.2) for group 2, p=0.08. The median operative time was 200 minutes (180-230) for group 1 and 203 minutes (120-300) for group 2, p=0.15. Major complications (Clavien ≥3) were 4 (5.6%) in group 1 and 1 (6.3%) in group 2, p=0.52. No deaths or early postoperative complications such as: urinoma or urinary leakage or bleeding, occurred. The success rate for radiologic improvement and flank pain improvement was comparable between the two groups. Re-garding hydronephrosis, significant improvement was present in 62 patients (93.4%) of group 1 and 10 cases (90.9%) of group 2, p=0.99. The median hospital stay was 4 days (IQR 3-4) for group 1 and 4.8 days (IQR 3-6) for group 2, p=0.27. CONCLUSIONS: Transperitoneal laparoscopic pyeloplasty is feasible and effective for the management of UPJO associated with renal or urinary tract anomalies.

Urethral duplication type influences on the complications rate and number of surgical procedures

ABSTRACT Introduction: Urethral duplication is rare. Characterized by the presence of two urethral channels. This anomaly presents a great variety of clinical findings that depend on the type of duplication that often is associated with other anomalies. Material and Methods: We report thirteen boys with urethral duplication managed in our institution between 1988-2015. Clinical findings, associated anomalies, treatment of urethral duplication and our results are described. Patients were classified according to Effmann classification. Results: Mean patient's age was 38.3±34.7 months (3-136 months). Mean follow-up was 7.7±3.4 years (3y8m-14y2m). Type II A2 was the most common pattern (8/13 patients, 61.5%), followed by type IA (3/13 patients, 23%) and IIA1 (2/13 patients, 15.3%). The most frequent clinical manifestations were urinary tract infections (UTI) observed in 11/13 patients (84.6%) and anal urinary leakage, found in 7/13 patients (53.8%). Associated anomalies were found in 9/13 patients (69.2%). Required surgeries were 3.53±2.84 procedures per patient. Considering groups: Type IIA2 4.25±3.28, type IIA1 4±1.41 and type IA 1.33±0.57 needed procedures per patient. Complications rate were 0% for type IA, 50% for type IIA1 and 75% for type IIA2. Conclusions: Patients with incomplete duplication (type I A or I B) can totally be asymptomatic, with no need of surgical correction. Type IIA2 is the most complex form of duplication to correct and multiple procedures might be required because of the very hypoplastic orthotopic dorsal urethral tissue. Surgical treatment should be individualized and parents should be advised on complications and need of multiple surgeries according to urethral duplication type.

Urethral duplication type influences on the complications rate and number of surgical procedures.

INTRODUCTION: Urethral duplication is rare. Characterized by the presence of two urethral channels. This anomaly presents a great variety of clinical findings that depend on the type of duplication that often is associated with other anomalies. MATERIAL AND METHODS: We report thirteen boys with urethral duplication managed in our institution between 1988-2015. Clinical findings, associated anomalies, treatment of urethral duplication and our results are described. Patients were classified according to Effmann classification. RESULTS: Mean patient's age was 38.3±34.7 months (3-136 months). Mean follow-up was 7.7±3.4 years (3y8m-14y2m). Type II A2 was the most common pattern (8/13 patients, 61.5%), followed by type IA (3/13 patients, 23%) and IIA1 (2/13 patients, 15.3%). The most frequent clinical manifestations were urinary tract infections (UTI) observed in 11/13 patients (84.6%) and anal urinary leakage, found in 7/13 patients (53.8%). Associated anomalies were found in 9/13 patients (69.2%). Required surgeries were 3.53±2.84 procedures per patient. Considering groups: Type IIA2 4.25±3.28, type IIA1 4±1.41 and type IA 1.33±0.57 needed procedures per patient. Complications rate were 0% for type IA, 50% for type IIA1 and 75% for type IIA2. CONCLUSIONS: Patients with incomplete duplication (type I A or I B) can totally be asymptomatic, with no need of surgical correction. Type IIA2 is the most complex form of duplication to correct and multiple procedures might be required because of the very hypoplastic orthotopic dorsal urethral tissue. Surgical treatment should be individualized and parents should be advised on complications and need of multiple surgeries according to urethral duplication type.

One - staged reconstruction of bladder exstrophy in male patients: long - term follow-up outcomes

ABSTRACT Introduction The surgical correction of bladder exstrophy remains challenging. In our institution, the repair has evolved from a staged repair to one-stage reconstruction. The one-stage reconstruction includes; bladder closure, Cantwell-Ransley neourethroplasty and abdominoplasty using groin flaps, without the need of pelvic ostheotomies. Repair of urinary continence (UC) and vesicoureteral reflux (VUR) is done after development of the infant. Objective To present our experience of our modified one-stage reconstruction of bladder exstrophy in male patients. Materials and Methods Medical records of male patients submitted to one-stage reconstruction of bladder exstrophy were analyzed retrospectively. Fifteen exstrophy bladder patients with mean age 4.2±7 years were treated at our institution between 1999-2013. Results 2 5 Conclusions One-stage reconstruction minimizes the number of surgical procedures required to achieve UC and potentiates bladder-neck function. The advantages of using groin flaps over current techniques for complete repair are the small risk for penile tissue loss and the avoidance of ostheotomies.

One - staged reconstruction of bladder exstrophy in male patients: long - term follow-up outcomes.

INTRODUCTION: The surgical correction of bladder exstrophy remains challenging. In our institution, the repair has evolved from a staged repair to one-stage reconstruction. The one-stage reconstruction includes; bladder closure, Cantwell-Ransley neourethroplasty and abdominoplasty using groin flaps, without the need of pelvic ostheotomies. Repair of urinary continence (UC) and vesicoureteral reflux (VUR) is done after development of the infant. OBJECTIVE: To present our experience of our modified one-stage reconstruction of bladder exstrophy in male patients. MATERIALS AND METHODS: Medical records of male patients submitted to one-stage reconstruction of bladder exstrophy were analyzed retrospectively. Fifteen exstrophy bladder patients with mean age 4.2±7 years were treated at our institution between 1999-2013. RESULTS: Eleven patients were referred to us after previous surgery. Sixteen procedures were performed; one patient had complete wound dehiscence and needed another reconstruction (6.7%). Mean follow up was 10.3±4.5 years. No patient has had a loss of renal function. Postoperative complications: four patients (26.6%) presented small fistulas, one presented penile rotation. Eleven patients (73.3%) patients underwent bladder-neck surgery. Five (33.3%) required bladder augmentation. Three cases (20%) needed subsequent treatment of VUR. At the time of our review nine (60%) patients achieved UC, two (13.3 %) patient without additional procedure. A mean of 3±1.1 procedures (2-5) was accomplished per children. CONCLUSIONS: One-stage reconstruction minimizes the number of surgical procedures required to achieve UC and potentiates bladder-neck function. The advantages of using groin flaps over current techniques for complete repair are the small risk for penile tissue loss and the avoidance of ostheotomies.

Step-by-step Laparoscopic Vesiculectomy for Hemospermia.

Hemospermia has been considered as a benign and self-limiting condition. It usually has an inflammatory or infectious cause. However, recurrent or persistent hemospermia may indicate a more serious underlying pathology, especially over 40 years of age. Biopsy or surgical excision is indicated in cases of suspicious findings during investigation, such as cysts or masses. Open surgery has been considered the definitive form of treatment, however, it can be associated with significant morbidity. With growing experience in laparoscopics, this approach is becoming the preferable way to access the seminal vesicles. Our objective is to demonstrate a step-by-step operative technique for laparoscopic unilateral vesiculectomy approach in a man with hemospermia. CASE: A 61 year-old man presented with 1 year of hemospermia. He was treated empirically with a fluoroquinolone plus a nonsteroidal anti-inflammatory without resolution of symptoms. Ultrasonography and MRI showed a solid-cystic mass in the right seminal vesicle. The patient was submitted to a laparoscopic unilateral vesiculectomy. Histopathological analysis showed intraluminal dilatation with blood content. During follow-up, complete resolution of symptoms was seen. RESULTS: Three patients composed our cohort. Mean age was 53 years-old (range 45-61 years), the right side was more commonly affected (two unilateral on the right and bilateral). Mean operative time was 55 minutes (range 40-120min). One patient presented amyloidosis in the histopathological analysis. All cases presented complete resolution of symptoms. CONCLUSIONS: Laparoscopic vesiculectomy is a safe and feasible approach in cases of hemospermia. This technique showed good outcomes and minimal morbidity.

Laparoscopic Pyeloplasty in children with Horseshoe Kidney.

INTRODUCTION: Horseshoe kidney occurs in 1 per 400-800 live births and are more frequently observed in males (M:F 2:1). Ureteropelvic junction obstruction (UPJO) is commonly associated with horseshoe kidneys. The variable blood supply, presence of the isthmus and high insertion of the ureter contribute to this problem. CASE REPORT: An asymptomatic 6 year-old boy presented with antenatal hydronephrosis. Ultrasonography and CT scan demonstrated left UPJO associated with a horseshoe kidney. DMSA showed 33% of function on the left side. DTPA showed a flat curve and lack of washout. A left dismembered laparoscopic pyeloplasty was performed after identification of crossing vessels and abnormal implantation of the ureter. After one year, the child is asymptomatic. DTPA demonstrated a good washout curve. RESULTS: Our cohort consisted of six patients, five males and one female, with a mean age of 6 years (range 6m-17 years) and a mean follow-up of 3 years. Ureteropelvic junction obstruction was more common on the left side. Symptoms appeared only in 34% of the cases. Mean operative time was 198 minutes (range 120-270 minutes). Crossing vessels were common (observed in 50% patients). High implantation of ureter was seen in 67% patients and intrinsic obstruction in 83%. Surgical difficulties were found in two cases. Hospital stay was 4.3 days (3 to 6 days), with only one patient having a mild complication (pyelonephritis). All cases had clinical and radiologic improvement. CONCLUSION: Laparoscopic pyeloplasty is safe and feasible in children with UPJO in horseshoe kidneys, with good results and minimal morbidity.

Reoperative Laparoscopic Ureteropelvic Junction Obstruction Repair in Children: Safety and Efficacy of the Technique.

PURPOSE: Failure after pyeloplasty for ureteropelvic junction obstruction in children may occur in up to 10% of cases. Therapeutic options include Double-J® stent placement, endoscopic treatment and reoperation. Laparoscopic and robotic reoperative modalities seem safe and efficacious, although pediatric series are limited in the literature. We report the largest known series of reoperative laparoscopic ureteropelvic junction obstruction repair in children and compare this approach to primary laparoscopic pyeloplasty. MATERIALS AND METHODS: We reviewed all children undergoing laparoscopic pyeloplasty at a single institution from 2004 to 2015. Reoperative laparoscopic ureteropelvic junction obstruction repair was compared to primary pyeloplasty. Groups were analyzed regarding demographics, operative time, complications, length of hospital stay and success, defined by improvement of symptoms, ultrasound and renogram. RESULTS: We identified 11 cases of reoperation (8 redo pyeloplasties and 3 ureterocalycostomies) and 71 primary pyeloplasties. Groups were not different in age, gender or weight. Median followup was 37 months. Median time between primary pyeloplasty and reoperation was 34 months. Median operative time was 205 minutes for the reoperative group and 200 for primary pyeloplasty (p = 0.98). Length of stay was longer in the reoperative group (p = 0.049), although no major complications were recorded in this group. All reoperative cases and 96% of primary pyeloplasty cases remained asymptomatic following surgery (p = 0.99). Postoperative improvement was similar for both groups on ultrasound (90% for reoperation vs 92% for primary pyeloplasty, p = 0.99) and renogram (80% vs 88%, p = 0.6). CONCLUSIONS: Laparoscopy seems to be safe and effective for management of failed pyeloplasty in children. Based on our data, reoperation is as safe and effective as primary pyeloplasty.

Clinical and haemodynamic evaluation of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy: Is schistosomiasis hypertension an important confounding factor?

INTRODUCTION: Chronic thromboembolic pulmonary hypertension is a disease affecting approximately 4,000 people per year in the United States. The incidence rate in Brazil, however, is unknown. The estimated survival for patients with chronic thromboembolic pulmonary hypertension without treatment is approximately three years. Pulmonary thromboendarterectomy for select patients is a potentially curative procedure when correctly applied. In Brazil, the clinical and hemodynamic profiles of chronic thromboembolic pulmonary hypertension patients have yet to be described. OBJECTIVES: To evaluate the clinical and hemodynamic characteristics of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy in a referral center for chronic thromboembolic pulmonary hypertension treatment in Brazil. METHODS: From December 2006 to November 2009, patients were evaluated and scheduled for pulmonary thromboendarterectomy. The subjects were classified according to gender, age and functional class and were tested for thrombofilia and brain natriuretic peptide levels. RESULTS: Thirty-five consecutive chronic thromboembolic pulmonary hypertension patients were evaluated. Two patients tested positive for schistosomiasis, and 31 were enrolled in the study (19 female, 12 male). The majority of patients were categorized in functional classes III and IV. Hemodynamic data showed a mean pulmonary vascular resistance (PVR) of 970.8 ± 494.36 dynas·s·cm-5 and a low cardiac output of 3.378 ± 1.13 L/min. Linear regression revealed a direct relation between cardiac output and pulmonary vascular resistance. Paradoxical septal movement was strongly correlated with pulmonary vascular resistance and cardiac output (p=0.001). Brain natriuretic peptide serum levels were elevated in 19 of 27 patients. CONCLUSIONS: In a referral center for pulmonary hypertension in Brazil, chronic thromboembolic pulmonary hypertension patients evaluated for pulmonary thromboendarterectomy had a hemodynamically severe status and had elevated brain natriuretic peptide serum levels. There was a predominance of females in our cohort, and the prevalence of hematological disorders and schistosomiasis was low (less than 10%).

Clinical and haemodynamic evaluation of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy: Is schistosomiasis hypertension an important confounding factor?

INTRODUCTION: Chronic thromboembolic pulmonary hypertension is a disease affecting approximately 4,000 people per year in the United States. The incidence rate in Brazil, however, is unknown. The estimated survival for patients with chronic thromboembolic pulmonary hypertension without treatment is approximately three years. Pulmonary thromboendarterectomy for select patients is a potentially curative procedure when correctly applied. In Brazil, the clinical and hemodynamic profiles of chronic thromboembolic pulmonary hypertension patients have yet to be described. OBJECTIVES: To evaluate the clinical and hemodynamic characteristics of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy in a referral center for chronic thromboembolic pulmonary hypertension treatment in Brazil. METHODS: From December 2006 to November 2009, patients were evaluated and scheduled for pulmonary thromboendarterectomy. The subjects were classified according to gender, age and functional class and were tested for thrombofilia and brain natriuretic peptide levels. RESULTS: Thirty-five consecutive chronic thromboembolic pulmonary hypertension patients were evaluated. Two patients tested positive for schistosomiasis, and 31 were enrolled in the study (19 female, 12 male). The majority of patients were categorized in functional classes III and IV. Hemodynamic data showed a mean pulmonary vascular resistance (PVR) of 970.8 ± 494.36 dynas·s·cm-5 and a low cardiac output of 3.378 ± 1.13 L/min. Linear regression revealed a direct relation between cardiac output and pulmonary vascular resistance. Paradoxical septal movement was strongly correlated with pulmonary vascular resistance and cardiac output (p=0.001). Brain natriuretic peptide serum levels were elevated in 19 of 27 patients. CONCLUSIONS: In a referral center for pulmonary hypertension in Brazil, chronic thromboembolic pulmonary hypertension patients evaluated for pulmonary thromboendarterectomy had a hemodynamically severe status and had elevated brain natriuretic peptide serum levels. There was a predominance of females in our cohort, and the prevalence of hematological disorders and schistosomiasis was low (less than 10 percent).