Clinical Improvement After Banding of a Pulmonary Branch Artery in a Symptomatic Patient with Osler–Rendu–Weber Syndrome

We report a symptomatic newborn with Osler–Rendu–Weber syndrome, multiple and diffuse pulmonary arteriovenous malformations, and right-to-left shunting in the left lung. Right-to-left shunting was significantly decreased by selectively banding the left pulmonary branch artery and clipping one large feeding vessel so that total resection of the left lung could be avoided.     

The Modified Blalock–Taussig Shunt Versus the Right Ventricle-to-Pulmonary Artery Conduit for the Norwood Procedure

The initial Norwood procedure remains the highest risk operation for the staged repair of univentricular congenital malformations with associated systemic outflow obstruction. The modified Blalock–Taussig shunt (MBTS) has been implicated as a major cause of not only the operative mortality, but also associated morbidity and interstage attrition. The etiology of these events has often been attributed to the diastolic runoff and “coronary steal” associated with the MBTS, in addition to the delicate balance between systemic and pulmonary blood flow that characterizes all systemic-to-pulmonary artery shunts. Recently, there has been renewed interest in the right ventricle-to-pulmonary artery conduit as a source of pulmonary blood flow for the Norwood procedure as a potential method for minimizing the negative aspects of the MBTS. The current literature is contradictory, retrospective, and predominantly historically controlled. The Trial of Right Ventricular vs Modified Blalock–Taussig Shunt in Infants with Single Ventricle Defect Undergoing Staged Reconstruction, a randomized controlled clinical trial comparing the two techniques, is ongoing and may provide answers to this controversy.     

Left Innominate Vein–Pulmonary Artery Shunt with Glenn Anastomosis in a Fontan Candidate with Central Pulmonary Artery Stenosis

Heart failure developed 9 years after Fontan takedown with systemic–pulmonary artery shunt in a 12-year-old girl with pulmonary atresia, intact ventricular septum, and obstruction in the proximal pulmonary artery. Surgical scar after multiple operations complicated direct repair of the pulmonary artery, and thus she was not eligible for definitive palliation. Left innominate vein–to–left pulmonary artery shunt using an expanded polytetrafluoroethylene conduit in association with Glenn anastomosis functionally established an unobstructive superior cavopulmonary connection without direct repair of the central pulmonary artery, later facilitating one and a half ventricle repair. Use of an extraanatomical shunt may functionally relieve central pulmonary artery obstruction in candidates for Fontan-like circulation.     

Intact Right Ventricle–Pulmonary Artery Shunt After Stage 2 Palliation in Hypoplastic Left Heart Syndrome Improves Pulmonary Artery Growth

For patients with hypoplastic left heart syndrome who have undergone the Norwood procedure with a right ventricle–pulmonary artery (RV-PA) shunt, the shunt can either be removed or left intact at the time of the stage 2 procedure. This study aimed to determine the effects of an intact shunt on pulmonary artery growth and clinical outcomes after the stage 2 procedure. A retrospective review of patients who underwent Norwood with an RV-PA shunt from 2005 to 2010 was performed. Catheterization data, echocardiographic data, postoperative outcome variables, and mortality data were collected. Pulmonary artery size was measured at pre-stage 2 and pre-Fontan catheterizations using the Nakata Index and the McGoon Ratio. Of the 68 patients included in the study, 48 had the shunt removed at the time of stage 2 (group 1), and 20 had the shunt left intact (group 2). The two groups did not differ in terms of pre-stage 2 hemodynamics or pulmonary artery size. After stage 2, group 2 had higher oxygen saturations. The two groups did not differ regarding duration of chest tube drainage, length of hospital stay, need for unplanned interventions, or mortality. Before Fontan, the group 2 patients had higher superior vena cava (SVC) pressures and more venovenous collaterals closed. There was increased pulmonary artery growth between the pre-stage 2 and pre-Fontan catheterizations in group 2 using both the Nakata Index (+148.5 vs ?52.4 mm²/m²; p = 0.01) and the McGoon Ratio (+0.36 vs +0.01; p = 0.01). These findings indicate that patients with an intact RV-PA shunt after stage 2 have greater pulmonary artery growth than patients with the shunt removed, with no increased risk of complications.     

Atypical Bland–White–Garland Syndrome with Stenosis of the Origin of the Left Coronary Artery: Catheter Intervention After Mammary Artery Bypass Stenosis and Residual Fistula to the Pulmonary Trunk

A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland–White–Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula and stenosis of the shunt at the implantation site developed that resulted in a dominant perfusion of the LCA from the right coronary artery. Interventional catheterization was performed with stenting of the LCA mammary artery anastomosis and subsequent coil occlusion of the residual fistula. After this intervention the LCA was exclusively perfused by the mammarian bypass with no residual leak to the pulmonary artery.     

Catheter Interventional Closure of a Large Right Pulmonary Artery–to–Left Atrial Communication in a Neonate

Right pulmonary artery?Cto?Cleft atrial communication is a rare congenital vascular malformation that results in a right-to-left shunting. This report describes the case history of a neonate with a large right pulmonary artery?Cto?Cleft atrial connection resulting in cyanosis and severe heart failure who underwent successful early catheter interventional therapy. In the neonate, this lesion can be diagnosed accurately using transthoracic echocardiography. Closure of the communication can be achieved even in infants via percutaneous interventional catheterization with a low procedure-related risk and a good midterm follow-up result.     

Branch Pulmonary Artery Growth After Blalock–Taussig Shunts in Tetralogy of Fallot and Pulmonary Atresia with Ventricular Septal Defect: A Retrospective, Echocardiographic Study

We performed a retrospective echocardiographic study in tetralogy of Fallot (TOF) or pulmonay atresia with ventricular septal defect (PA&VSD) to evaluate the effects of Blalock–Taussig shunt on branch pulmonary artery growth. There were 35 patients with TOF and 11 with PA&VSD. We measured the right and left pulmonary artery area index and also the combined pulmonary artery area index, both before and after shunt operation. The mean ± SD of these three variables before the shunt operation in the TOF group were 63.5 ± 22.5, 57.8 ± 24.9, and 121.4 ± 42.8 mm²/m²; after shunt operation they were 98.5 ± 33.6, 85.9 ± 31.9, and 184.0 ± 59.8 mm²/m², respectively (p values <0.0001, <0.0002, and <0.0001, respectively). In the PA&VSD group the comparable values before shunt operation were 66.5 ± 16.0, 55.4 ± 10.6, and 120.9 ± 26.9 mm²/m² and after shunt operation were 90.5 ± 22.9, 77.2 ± 24.1, and 166.6 ± 44.4 mm²/m², respectively (p values <0.0006, <0.014, and <0.002, respectively). We also examined the effect of distensibility of pulmonary arteries by comparing the percentage change in size of the combined pulmonary artery area index in the first 4 months after shunt with those after this time (p < 0.023). There were no significant differences between left- and right-sided shunts, origin and distal pulmonary artery growth, and the TOF and PA&VSD groups.     

Tetralogy of Fallot with Pulmonic Atresia with Cyclic Occlusion of an Associated Aortic Sinus of Valsalva–Pulmonary Artery Window

A first case of an unusual aortopulmonary window with tetralogy of Fallot associated with pulmonary atresia is presented. The aortopulmonary window was at the aortic sinus of Valsalva. The left aortic leaflet prevented pulmonary hypertension by occluding the window in systole.     

Is Tracheoplasty Necessary for All Patients With Pulmonary Artery Sling and Tracheal Stenosis?

Pulmonary artery sling frequently accompanies various degrees of tracheal stenosis. The authors reviewed their surgical outcomes for pulmonary artery sling from two institutions. From 1997 to September 2011, 16 patients with pulmonary artery sling underwent surgical treatment. Their median age was 4.6 months and their body weight 6.2 kg at the time of the operation. Of the 16 patients, 12 showed preoperative respiratory symptoms such as recurrent airway infection or dyspnea, with three of these patients receiving ventilator therapy preoperatively. Most of the patients showed various degrees of tracheal luminal stenosis and stenotic length, except for three patients who did not show significant airway stenosis. Left pulmonary artery reimplantation was performed for all the patients, but an accompanying tracheoplasty was performed for only two patients (one patch augmentation and one sliding tracheoplasty). After surgery, early extubation and intensive lung care were performed. Two operative mortalities occurred: one due to cardiac tamponade associated with postoperative bleeding and one due to aggravation of preexisting multiorgan failure. Two late mortalities occurred: one due to airway obstruction by repetitive granulation tissue growing at the tracheoplasty site and one due to pneumonia. The 12 living patients, including 11 patients who did not undergo tracheal surgery, showed no clinically significant airway problems at their last follow-up visit. The surgical outcomes for pulmonary artery sling without tracheoplasty were acceptable. The authors think tracheoplasty may be avoided by intensive postoperative airway management for a significant portion of the patients with pulmonary artery sling and tracheal stenosis.     

Assessing Pulmonary Arterial Hypertension in Infants With Severe Chronic Lung Disease of Infancy: A Role for a Pulmonary Artery Catheter?

The treatment of pulmonary arterial hypertension (PAH) associated with chronic lung disease of infancy (CLDI) is becoming commonplace. However, an optimal approach to the monitoring of this treatment has not been clearly established, and data suggest that such therapy may not be without risk. This study assessed the feasibility and safety of pulmonary artery catheter (PAC) placement and its role in the management of PAH associated with CLDI. The medical records of 12 infants with CLDI requiring chronic mechanical ventilation who underwent PAC monitoring were reviewed. Data analyzed included demographics, hemodynamic data, PAH pharmacological therapy, respiratory support, echocardiographic data, sedation level, complications related to PAC use, and mortality. In this analysis, PAC placement and monitoring was found to be feasible, appeared safe, and was associated with the ability to wean inspired oxygen, decrease sedation, and titrate PAH therapy without untoward effect. However, no definitive conclusions can be drawn from this report given its small sample size and uncontrolled, retrospective design. It is hoped that these data will renew interest in PAC monitoring for CLDI and foster prospective study where its true value can be ascertained.     

Pulmonary Functions in Normal School Children in the Age Group of 6–15 Years in North India

Objective

Lung function tests have become an integral part of assessment of pulmonary disease. As few studies on pulmonary function tests have been carried out in young children in India, the study was carried out in normal urban and rural school children in Ludhiana district of North India to determine pulmonary functions in the age group 6–15 years and to find its correlation with regards to age, sex, height and weight.

Methods

The study group included 600 normal children between 6–15 years age from different urban and rural schools in the region. A preformed questionnaire was interviewed and detailed general physical and systemic examination was done. Pulmonary function tests were measured by using Micromedical Gold standard fully computerized portable auto spirometer (Superspiro Cat No. SU 6000).

Findings

The present study shows, all the three independent variables (age, weight and height) have linear positive correlation with lung function parameters, both for boys and girls. Lung function values in boys were significantly higher as compared to that of girls. Urban children had higher lung function parameters than rural children except IRV, FEF_25%. Among all anthropometric parameters, height was the most independent variable with maximum coefficient of correlation.

Conclusion

Equations derived from the present study for estimation of the expected values of lung function will help to interpret the observed lung function values in children of North India.     

Outpatient Visits Before Ambulatory Care–Sensitive Hospitalization of Children Receiving Medicaid

Objectives

Hospitalizations for ambulatory care–sensitive conditions (ACSC) are measured to indicate health care system quality, with the premise that fewer hospitalizations would occur with better preceding outpatient care. Our objectives were to identify outpatient care received in the 7 days preceding acute pediatric hospitalizations and to compare receipt of outpatient care by hospitalization type (ACSC vs non-ACSC).

Persistent Interstitial Pulmonary Emphysema–like Cyst Associated with Metastatic Synovial Sarcoma

Interstitial pulmonary emphysema is characterized by the presence of gas dissecting the interstitial tissues of the lung. Clinically, it may be acute or persistent, and the latter can be further categorized as localized or diffuse. Usually, it appears in preterm neonates with a history of assisted ventilation or respiratory distress. Although far from frequent, the localized variety of persistent interstitial pulmonary emphysema (PIPE) can develop spontaneously in full-term babies or infants without any obvious underlying pulmonary disease. Histologically, PIPE is characterized by the presence of uni- and multinucleated histiocytes lining the inner surface of the cysts. In this report, we describe a 15-year-old male with synovial sarcoma (SS) of the right ankle diagnosed 4 years previously who developed pulmonary metastasis, one of which presented cystic changes with features of PIPE. Received April 2, 1999; accepted July 23, 1999.     

Interventions After Norwood Procedure: Comparison of Sano and Modified Blalock–Taussig Shunt

Improved results have evolved from the modified Norwood procedure (NP). This study compares the incidence of interventions after NP with the Sano (n = 37) and modified Blalock–Taussig (BT n = 70) shunt. Incidence, location, interval of interventions, and weight were retrospectively analysed for 107 neonates undergoing NP during the period from October 2002 to December 2009. Forty-six (43.0 %) patients underwent interventions, mostly for dilatation of the aortic arch ([DAA] n = 26 [24.3 %]; Sano n = 10, BT n = 16, p = 0.6), dilatation of the shunt ([DS] n = 15 [14.0 %]; Sano n = 11, BT n = 4; p = 0.002), or closure of aortopulmonary collaterals ([APC] n = 15 [14.0 %]; Sano n = 3, BT n = 12; p = 0.08). Mean interval after NP and body weight at DAA, DS, and APC were 72.4 ± 18.9, 108.5 ± 15.8, and 110.7 ± 17.8 days and 4.5 ± 1.3, 4.9 ± 1.9, 5.3 ± 1.2 kg, respectively. The interventions were not associated with mortality but with a greater rate of complications (9 of 46 [21.4 %]) compared with the rate after diagnostic catheterization (0 of 45, p = 0.03). Complications included closure of the femoral or subclavian artery (n = 5), cerebral embolic or bleeding events (n = 4), cardiopulmonary resuscitation (n = 3), and temporary heart block (n = 2). Actuarial survival was similar from the postoperative month 8 onward at 78.6 ± 4.9 % (95 % confidence interval [CI] 67.0–86.5 %) for Sano and 78.4 ± 6.8 % (95 % CI 61.4–88.6 %) for BT (p = 0.95). Interventions after NP were common irrespective of shunt type. However, a significantly greater rate of shunt interventions was noted in the Sano group. In particular, interventions addressing the aortic arch and the shunt were related with a significant rate of complications.     

Paclitaxel Drug–Eluting Stent Placement for Pulmonary Vein Stenosis as a Bridge to Heart–Lung Transplantation

Congenital pulmonary vein stenosis (PVS) presents as an isolated lesion or in association with other congenital heart anomalies. The most extensive forms of the disease are uniformly fatal because neither surgical repair nor transcatheter therapy results in long-term relief of the stenosis. A case is presented involving single-ventricle physiology associated with extensive and recurrent congenital PVS despite multiple attempts with surgical therapy. Heart–lung transplantation was ultimately performed after drug-eluting stents were placed in pulmonary veins as a bridge to the transplantation.     

Dysphagia Lusoria Caused by Aberrant Right Subclavian Artery, Kommerell’s Diverticulum, Legamentum Ring, Right Descending Aorta, and Absent Left Pulmonary Artery: A Report of a Unique Vascular Congenital Disease Undetected until Adulthood and a Review of the Literature

An active otherwise healthy and middle-aged woman presented with left supraclavicular pulsation, right upper extremity claudication, and mild dysphagia. Evaluation revealed an aberrant right subclavian artery, Kommerell’s diverticulum with aneurysmal degeneration, legamentum arteriosum completing vascular ring, and absent left pulmonary artery with multiple collateral supply to the left lung. She underwent successful surgical repair via right thoracotomy, including division of the vascular ring, resection of the diverticulum and aneurysm, and finally reimplantation of the right subclavian artery to the aortic arch. Her symptoms resolved completely, and she was able to resume normal activities.     

Surgical implications of the Smith–Lemli–Opitz syndrome

Smith–Lemli–Opitz syndrome (SLOS) is a syndrome of rare multiple congenital anomalies/mental retardation associated with low plasma cholesterol levels. Prior to receiving the diagnosis of SLOS, affected children may present as a neonatal surgical emergency with ambiguous genitalia, Hirschsprungs disease, and pyloric stenosis. We present two fatal cases of SLOS with near-total Hirschsprungs disease; the surgical, anaesthetic, and medical aspects of the cases are discussed, and a literature review is presented.