Cost-Effectiveness Analysis of Stents,Balloon Angioplasty,and Surgery for the Treatment of Branch Pulmonary Artery Stenosis

Branch pulmonary artery stenosis is a common problem in pediatric cardiology. Treatment has included surgery, balloon angioplasty, and balloon expandable stent placement. It was the purpose of this investigation to demonstrate the cost-effectiveness of each of these modes of treatment. From 1983 to 1994 there were 30 patients admitted for treatment of branch pulmonary artery stenosis only. Data included age at procedure, sex, primary diagnosis, acute and intermediate term success, and complications. Acute success was defined by results at the end of the procedure where intermediate term (IT) success was defined by results at follow-up. Success of a procedure was defined by at least one of the following: an increase in vessel diameter by ≥50% of predilation diameter, a decrease in right ventricular to left ventricular or aortic systolic pressure ratio by ≥20%, or a decrease in peak to peak pressure gradient by ≥50%. The procedure was considered a failure if the previously mentioned criteria were not met or if the patient required a second procedure for the same stenosis. The expense of the procedure (estimated by using the patient charges) were collected from the time of the procedure until December 1994. Because of differing lengths of follow-up, the patients were analyzed separately for procedures and outpatient charges. The total charges were corrected to 1994 dollars using the Medical Consumer Price Index. Thirty patients had 46 separate procedures (12 patients had >1 procedure and 3 had >2 procedures). There were 13 surgeries, 13 balloon angioplasties, and 20 stents. Stents were the most successful (90% acute and 85% IT), but were not statistically superior to surgery (62% acute and IT). Balloon angioplasty was significantly less successful as compared with stents (31% acute and 23% IT), and was not statistically different from surgery over the acute and intermediate term. The charge data showed balloon angioplasty was the least expensive followed by stents and then by surgery. The average total charges per procedure, including outpatient charges, were: surgery $58,068 ± $4372 (standard error), balloon $21,893 ± $5019, stents $33,809 ± $3533 (p < 0.001); excluding outpatient charges: surgery $52,989 ± $3649, balloon $15,653 ± $1691, and stents $29,531 ± $2241 (p < 0.001). Average total charges per patient, including all procedure types and grouped by initial procedure, were: surgery $53,707 ± $6388, balloon $50,040 ± $8412, and stent $34,346 ± $3488 (p= 0.047). Stents were at least as effective as surgery and were more effective than balloon angioplasty in both acute and intermediate term follow-up. Balloon angioplasty was least expensive per procedure but was also least effective. Therefore, intravascular balloon expandable stents are the most cost-effective means available in the treatment of branch pulmonary artery stenosis.     

Pulmonary Stenosis is a Predictor of Unfavorable Outcome After Surgery for Supravalvular Aortic Stenosis

We sought to evaluate whether the presence of pulmonary stenosis (PS), amongst other factors, influences the mortality and the rate of reoperations in the long-term follow-up of patients with supravalvular aortic stenosis (SVAS). We identified all patients with SVAS from our surgical database. The patients with multi-level aortic stenosis or concomitant cardiac procedures were excluded from this study. Follow-up (100?%) was conducted between 2008 and 2010. Twenty-six patients underwent surgery for SVAS between 1974 and 2006. Seventeen patients (65?%) were diagnosed with Williams-Beuren-Syndrome, six (17?%) had a diffuse form of SVAS and 10 (39?%) had PS. No patient had a surgical or interventional procedure for PS at the initial operation or during follow-up. There was no statistically significant association between PS and WBS (p?=?0.30) or diffuse form of SVAS (p?=?0.13). Patients with PS were operated at younger age (p?=?0.028). Median follow-up time was 14.6?years. Overall mortality was 11.5?%. One patient with preoperatively severely decreased LV-function died 27?days postoperatively. Two late deaths occurred 7 and 10?years after the initial operation. Reoperations were required in 4 patients (15?%), 4-19?years after the original operation, due to aortic arch stenosis, supravalvular restenosis or poststenotic aortic dilatation. PS was found to be a risk factor for reoperation (p?=?0.005) and for the combined reoperation/death end-point (p?=?0.003). PS in patients with SVAS is a risk factor for reoperations in the aortic region and might be considered an indicator of the severity of the arterial disease and a predictor of an unfavourable outcome.     

A Case of Left Main Pulmonary Artery Aneurysm Associated with Valvular Pulmonary Stenosis in a Child

Aneurysm of the main pulmonary artery is a rare clinical entity that can be congenital or acquired. Most cases occur in association with other congenital malformations, severe pulmonary hypertension, vasculitides, infectious agents, or collagen vascular disorders. We report here a pediatric case of left pulmonary artery aneurysm associated with valvular pulmonary stenosis and a hypoplastic right pulmonary artery, which we confirmed via multidetector computed tomography angiography.     

Long-Term Outcome of Sirolimus-Eluting Stent Implantation for Left Main Coronary Artery Stenosis in Infancy

Drug-eluting stents (DES) have markedly reduced the incidence of coronary in-stent restenosis. However, DES may be still vulnerable to coronary thrombus, and the long-term outcome remains unknown, especially in infancy. Here, we present a 9-month-old infant, who developed severe stenosis of the left main coronary artery after surgery for Brand-White-Garland syndrome, and was successfully treated with DES. He was healthy and his cardiac function had improved to the normal level at 6 years old. An angiographic examination and computed tomography showed the complete persistence of DES and no evidence of intimal thickening.     

Clinical Outcome,Valve Dysfunction,and Progressive Aortic Dilation in a Pediatric Population With Isolated Bicuspid Aortic Valve

The aim of this study was to explore the medium-term clinical outcome and the risk of progression of aortic valve disease and aortic dilation in pediatric patients with isolated bicuspid aortic valve (BAV). 179 pediatric patients with isolated BAV were prospectively followed from January 1995 to December 2010. Patients with severe valve dysfunction at baseline were excluded. Clinical outcome included cardiac death, infective endocarditis, aortic complications, cardiac surgery and percutaneous valvuloplasty. Echocardiographic endpoints were: progression of aortic stenosis (AS) or regurgitation (AR) and progressive aortic enlargement at different levels of the aortic root, evaluated as z-score. The median age at diagnosis was 7.8 [2.7–12.0] years. After a median followup of 5.4 [2.3–9.2] years, all patients were alive. The clinical endpoint occurred in 4 (2.2 %) patients (0.41 events per 100 patient-years). A progression of AS and AR was observed in 9 (5.0 %) and 29 (16.2 %) patients, respectively. The z-scores at the end of follow-up were not significantly different from baseline at the annulus, Valsalva sinuses and sinotubular junction, whereas a slight increase was observed at the level of the ascending aorta (1.9 vs 1.5, p = 0.046). Significant progressive aortic dilation occurred in a minority of patients (10.6, 5.6, 9.5, and 19.0 % respectively). The clinical outcome in pediatric patients with isolated BAV is favourable and the progression of aortic valve dysfunction and aortic dilation is relatively slow. These findings may be taken into account to better guide risk assessment and clinical follow-up in these patients.     

Clinical Manifestations and Long-Term Follow-Up in Pediatric Patients Living at Altitude With Isolated Pulmonary Artery of Ductal Origin

This study's aim was to define the clinical manifestations and long-term outcome of pediatric patients living at altitude with isolated pulmonary artery (PA) of ductal origin (IPADO). This was a retrospective cohort study of 17 consecutive cases of IPADO at a single center. All patients lived at modest altitude (median 2050 m [range 1700 m to 3050 m]). Fifteen children (88%) were symptomatic at presentation. High-altitude pulmonary edema was present in 2 patients (12%) at diagnosis, and only 1 patient had episodes of hemoptysis during follow-up. Fourteen patients (82%) demonstrated evidence of pulmonary arterial hypertension (PAH). Among 14 patients with PAH, 11 patients had surgical interventions. PAH resolved in 5 of 11 patients (45%) undergoing surgical rehabilitation. One patient died during follow-up, and 7 patients are receiving oral vasodilator therapies due to residual PAH; 14 patients remained asymptomatic. Our study showed that early intervention in patients with IPADO at modest altitude can potentially rehabilitate the isolated PA and reverse PAH. Whether surgery is indicated for patients with this disorder in the absence of PAH is unknown.     

Tetralogy of Fallot With Atrioventricular Septal Defect: Surgical Strategies for Repair and Midterm Outcome of Pulmonary Valve-Sparing Approach

Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has been reported with good early and intermediate outcomes. Morbidity, however, remains significantly high. To date, repair of CAVSD/TOF using a pulmonary valve-sparing technique (PVS) and freedom from valve reoperation are not well defined. A study was undertaken to investigate outcomes. This study was conducted in as a retrospective investigation. Between January 1988 and December 2008, 13 consecutive patients with CAVSD/TOF were identified, and their records were reviewed retrospectively. Of these 13 patients, 9 had Rastelli type C CAVSD. Trisomy 21 was present in 9 cases (69 %; 7 with type C). Five patients had received a systemic-to-pulmonary shunt (SPS) before complete repair at a mean age 1.7 ± 0.6 months. All the patients survived until complete repair. At complete CAVSD/TOF repair, AVSD was corrected with a two-patch technique in all patients. For eight patients (61.5 %), PVS was used. The remaining five patients had transannular patch (TAP) repair. The mean age at complete repair was 6.3 ± 2.4 months. At complete repair, the mean cardiopulmonary bypass time was 173.5 ± 30.6 min, and the cross-clamp time was 134.7 ± 28.8 min. There was one hospitalization and no late deaths. The median follow-up period was 9.2 years [interquartile range (IQR), 4.7–13.3 years]. The actuarial survival was 90.0 ± 9.5 % at 1 year, 90 ± 9.5 % at 5 years, and 90 ± 9.5 % at 8 years. Of the 12 survivors, 6 had some reintervention during the follow-up period. Within the first 11 years after complete repair, two patients underwent left atrioventricular (AV) valve repair, and one patient had right AV valve repair. Two patients had residual VSD closure. Four patients underwent the first right ventricular outflow tract (RVOT) reintervention for critical insufficiency or stenosis at a mean interval of 6 ± 21) months. One patient had a second RVOT reoperation. Findings showed that CAVSD/TOF with PVS was related to significantly higher freedom from RVOT reintervention (100 % at 1, 5, and 8 years compared with 80 ± 17.9 % at 1 year, 60 ± 21.9 % at 5 years, and 40 ± 21.9 % at 8 years for CAVSD/TOF using TAP; P < 0.05). No patient who underwent PVS had left ventricular outflow tract obstruction requiring reoperation. Overall freedom from any reintervention was 90.9 ± 8.6 % at 1 year, 71.6 ± 14.0 % at 5 years, and 53.7 ± 8.7 % at 8 years in this group of patients. Correction of TOF with CAVSD can be performed at low risk with favorable intermediate-term survival and satisfactory freedom from reoperation. Use of TAP can be avoided in almost two thirds of patients and may influence freedom from early RVOT reintervention.     

Autologous Right Pulmonary Artery Tissue for Repair of Left Pulmonary Artery Originating from Left Patent Ductus Arteriosus in a Patient With Tetralogy of Fallot and Absent Pulmonary Valve

Left pulmonary artery (PA) originating from patent ductus arteriosus is an exceptionally rare variant of tetralogy of Fallot with absent pulmonary valve. We described an alternative technique for the repair of discontinuous left PA with the use of the redundant pulmonary artery tissue as material for the conduit in a 3-year-old boy.     

Bronchial Compression as Adverse Effect of Left Pulmonary Artery Stenting in a Patient with Hypoplastic Left Heart Syndrome

A 5-year-old child with hypoplastic left heart syndrome (HLHS) was noted to have left pulmonary artery stenosis after Glenn shunt placement. Therefore, a stent was implanted into the left pulmonary artery to relieve stenosis. After redilatation of the pulmonary artery stent, wheezing, fatigue, asymmetric bronchial sound, and desaturation was noted. Computed tomography, bronchoscopy, and bronchography confirmed critical left main bronchus compression as a consequence of the mass effect of the dilated stent. Balloon plasty of the left bronchus successfully relieved bronchial stenosis. Tracheobronchial obstruction is a possible adverse effect of vascular stenting in children with congenital heart defects. Balloon plasty seems to be a safe and efficient therapeutic option in those cases.     

Coronary Artery-to-Pulmonary Artery Fistula in a Case of Pulmonary Atresia With Ventricular Septal Defect

Pulmonary atresia with ventricular septal defect (PA-VSD) can be associated with varying pulmonary artery connections. The origin of pulmonary blood flow can vary greatly among patients, and some case reports have described the presence of left coronary artery-to-pulmonary artery fistula. Two cases of patients found to have coronary artery-to-pulmonary artery fistula in the settings of PA-VSD are reported.     

Stenting of the Aorta for Recurrent, Long Stenosis Due to Takayasu's Arteritis in a Child

Balloon angioplasty followed by stenting of the thoracic aorta is reported in a 5-year-old girl with Takayasu's arteritis, who presented with severe hypertension and congestive heart failure. Her aortogram showed severe long segment stenosis of the descending thoracic aorta, which was successfully treated by balloon angioplasty on two occasions, but developed recurrences after 6 and 7 months of angioplasty. Balloon dilatation of the stenosed aorta, followed by sequential implantation of three 30-mm long Palmaz stents in tandem, bridging the full segment of the angioplastied aorta were performed successfully without any complications. The aortic diameter increased from 3.3 mm to 7.7 mm and the peak systolic pressure gradient decreased from 75 mmHg to 3 mmHg. There was no recurrence on clinical follow-up of 13 months.     

Percutaneous Coronary Intervention for Acute Myocardial Infarction in a Pediatric Patient With Coronary Aneurysm and Stenosis Due to Kawasaki Disease

We report the case of an acutely ill 3-year-old female, with a previous medical history of Kawasaki disease, who presented to care with an acute myocardial infarction. We describe the coordinated therapies employed by pediatric and adult cardiologists aimed to establish coronary revascularization.     

Benign Outcome of Pulmonary Hypertension in Neonates With a Restrictive Patent Foramen Ovale Versus Result for Neonates With an Unrestrictive Patent Foramen Ovale

Premature closure or restriction of foramen ovale (FO) is a rare but known entity. FO diameter <2 mm and Doppler velocity >120 cm/s, diameter <3 mm with Doppler velocity measured gradient >5 mmHg have all being used by various authors to describe this entity. Some neonates with restrictive FO have been noted to have severe pulmonary hypertension with no clinical signs or symptoms and with spontaneous resolution without any intervention. Seven consecutive neonates were indentified in the database between 01/01/2003 and 06/30/2010 with diagnosis of restrictive PFO (diameter <2 mm) with structurally normal heart and their initial and follow-up echocardiogram as well as hospital medical records were reviewed. As a control, seven neonates with diagnosis of pulmonary hypertension and respiratory distress syndrome or meconium aspiration syndrome were randomly selected. Eighty-six percent of the patients in the control group were symptomatic and required treatment as compared to 14% in the restrictive FO group (p = 0.03). Further, the fall in the peak instantaneous pulmonary artery pressure on follow-up echocardiogram was greater in the restrictive FO group compared with the non-restrictive group (p = 0.03). Patients with pulmonary hypertension and a restrictive FO with no other associated congenital heart disease and/or lung pathology behave differently when compared to neonates with non-restrictive FO and pulmonary hypertension with associated lung disease. They seldom manifest symptoms requiring intervention and tend to show a faster drop in their pulmonary artery pressure toward the normal.     

Successful Dilatation of Left Pulmonary Artery Stenosis by Stent Implantation Through a Modified Blalock-Taussig Shunt in an Infant with Pulmonary Atresia and Ventricular Septal Defect

We successfully implanted a coronary stent via a modified Blalock-Taussig shunt to relieve stenosis of the left pulmonary artery in an infant with pulmonary atresia and ventricular septal defect. The placement of a flexible coronary stent via a modified Blalock-Taussig shunt is a safe and feasible catheter intervention for the treatment of infants with hypoplastic pulmonary arteries.     

Correlation of Subjective Questionnaires With Cardiac Function as Determined by Exercise Testing in a Pediatric Population

Background: Although exercise testing is an important objective method used to assess cardiopulmonary function, subjective assessment often is used as a proxy in the clinical setting. This study aimed to determine whether responses to a subjective functional capacity questionnaire administered to parents and patients in a pediatric exercise laboratory correlate with objective assessment of functional capacity, measured by peak oxygen consumption during maximal voluntary exercise testing. Methods: Subjective questionnaire responses and exercise test results collected over 10 years were retrospectively analyzed. Symptoms and physical capabilities were assessed using a 6-point Likert scale regarding the ability to attend school/work, walk, climb stairs, and run, as well as the frequency of fatigue, palpitations, and chest pain. Values of 0 to 3 were considered abnormal, whereas values of 4–5 were regarded as normal. Exercise testing was performed on a stationary cycle ergometer with a continuous ramping protocol. Heart rate and oxygen saturation were continuously monitored. Blood pressures and electrocardiograms (ECGs) were obtained at 2-min intervals. Metabolic gas analysis was performed using a breath-by-breath method. The results of the exercise testing were normalized for body size and expressed as a percentage of predicted peak oxygen consumption (%pVO₂). Results: Very weak but statistically significant correlations (τ < 0.25; P < 0.05) between the scores of the school/work, walking, stair climbing, running, and fatigue items and %pVO₂ were found using Kendall’s rank correlations. Conclusions: The subjective Likert scales used to assess basic physical capacity and cardiac-associated symptoms have limited ability to predict actual functional capacity as measured by %pVO₂ achieved. The very weak rank-order correlation between %pVO₂ achieved and the subjective reporting of the ability to attend school/work, walk, climb stairs, and run has low clinical significance and will not be useful in predicting functional capacity within the clinic setting.