Restrictive interatrial communication after reconstructive surgery for hypoplastic left heart syndrome.
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Adolescent↗
Biomedical subjects
T L Spray
Publications and source records attributed to T L Spray.
Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled ventilation.
BACKGROUND: Management strategies for preoperative infants with hypoplastic left heart syndrome (HLHS) include increased inspired nitrogen (hypoxia) and increased inspired carbon dioxide (hypercarbia). There are no studies directly comparing these 2 therapies in humans. This study compares the impact of hypoxia versus hypercarbia on oxygen delivery, under conditions of fixed minute ventilation. METHODS AND RESULTS: Ten anesthetized and paralyzed preoperative infants with HLHS were evaluated in a prospective, randomized, crossover trial comparing hypoxia (17% FIO(2)) with hypercarbia (2.7% FICO(2)). Each patient was treated in a random order (10 minutes per condition) with a recovery period (15 to 20 minutes) in room air. Arterial (SaO(2)) and superior vena caval (SvO(2)) co-oximetry and cerebral oxygen saturation (ScO(2)) measurements were made at the end of each condition and recovery period. ScO(2) was measured by near infrared spectroscopy. Hypoxia significantly decreased both SaO(2) (-5.2+/-1.1%, P=0.0014) and SvO(2) (-5.6+/-1.7%, P=0.009) compared with baseline, but arteriovenous oxygen saturation (AVO(2)) difference (SaO(2)-SvO(2)) and ScO(2) remained unchanged. Hypercarbia decreased SaO(2) (-2.6+/-0.6%, P=0.002) compared with baseline but increased both ScO(2) (9.6+/-1.8%, P=0.0001) and SvO(2) (6+/-2.2%, P=0.022) and narrowed the AVO(2) difference (-8.5+/-2.3%, P=0.005). Both hypoxia and hypercarbia decreased the balance between pulmonary and systemic blood flow (Qp:Qs) compared with baseline. CONCLUSIONS: In preoperative infants with HLHS, under conditions of anesthesia and paralysis, although Qp:Qs falls in both conditions, oxygen delivery is unchanged during hypoxia and increased during hypercarbia. These data cannot differentiate cerebral from systemic oxygen delivery.
Administration, Inhalation↗
Are outcomes of surgical versus transcatheter balloon valvotomy equivalent in neonatal critical aortic stenosis?
BACKGROUND: For neonates with critical aortic valve stenosis who are selected for biventricular repair, valvotomy can be achieved surgically (SAV) or by transcatheter balloon dilation (BAV). METHODS AND RESULTS: Data regarding 110 neonates with critical aortic valve stenosis were evaluated in a study by the Congenital Heart Surgeons Society from 1994 to 1999. Reduced left ventricular function was present in 46% of neonates. The initial procedure was SAV in 28 patients and BAV in 82 patients. Mean percent reduction in systolic gradient was significantly greater with BAV (65+/-17%) than SAV (41+/-32%; P<0.001). Higher residual median gradients were present in the SAV versus BAV group (36 mm Hg [range, 10 to 85 mm Hg] versus 20 mm Hg [0 to 85 mm Hg], P<0.001). Important aortic regurgitation was more often present after BAV (18%) than SAV (3%; P=0.07). Time-related survival after valvotomy was 82% at 1 month and 72% at 5 years, with no significant difference for SAV versus BAV, even after adjustment for differences in patient and disease characteristics. Independent risk factors for mortality were mechanical ventilation before valvotomy, smaller aortic valve annulus (z score), smaller aortic diameter at the sinotubular junction (z score), and a smaller subaortic region. A second procedure was performed in 46 survivors. Estimates for freedom from reintervention were 91% at 1 month and 48% at 5 years after the initial valvotomy and did not differ significantly between groups. CONCLUSIONS: SAV and BAV for neonatal critical aortic stenosis have similar outcomes. There is a greater likelihood of important aortic regurgitation with BAV and of residual stenosis with SAV.
Acute Disease↗
Unexpected death after reconstructive surgery for hypoplastic left heart syndrome.
BACKGROUND: Although operative mortality for reconstructive surgery for hypoplastic left heart syndrome continues to improve, nonoperative mortality, especially in the first year of life, remains relatively high. A number of patients who are thought to be clinically well at hospital discharge die unexpectedly. The goal of the present study was to determine the incidence of and risk factors for unexpected death in patients with hypoplastic left heart syndrome. METHODS: Retrospectively, we determined the incidence of unexpected death among 536 patients with hypoplastic left heart syndrome who were discharged to home after stage I surgical procedure. To identify potential risk factors, a nested case-control analysis was undertaken. RESULTS: Unexpected death occurred in 22 of 536 patients (4.1%) discharged to home after stage I surgical procedure. The median age at unexpected death was 79 days (range, 25 to 227 days). Seizures preceded cardiac arrest in 2 patients, and ventricular arrhythmias were documented in 3 additional patients during attempted resuscitation. Autopsy studies were performed in 12 patients and identified residual lesions that may have contributed to death in 2 patients. In multivariate analysis documented perioperative arrhythmia and earlier year of stage I surgical procedure were associated with an increased risk for unexpected death (p = 0.03 and p = 0.04, respectively). There were 4 additional patients who had unexpected death after subsequent cavopulmonary operation at a median age of 1.6 years (range, 0.9 to 3.8 years). CONCLUSIONS: Unexpected death occurred in more than 4% of patients with hypoplastic left heart syndrome who were discharged to home after stage I surgical procedure and was most common in the first several months of life. Factors that may contribute to unexpected death include residual lesions, arrhythmias, and neurologic events, although in the majority of cases the cause remains largely unknown.
Cardiac Surgical Procedures↗
Epicardial atrial pacemaker lead placement after multiple cardiac operations.
We describe a simple technique for the implantation of left atrial epicardial pacing leads in children with congenital heart disease who have undergone multiple operations. The pulmonary veins are exposed to reveal the pulmonary venous to atrial confluence using a left thoracotomy. A pacemaker lead is secured to the posterior left atrium inferior to the lower pulmonary vein. This approach provides a reliable site for atrial lead placement without the need for extensive dissection.
Child↗
Thrombus formation after the Fontan operation.
BACKGROUND: Thrombus formation is common after a Fontan operation. We investigated the frequency and location of thrombus in our population of children based on the type of Fontan operation performed. METHODS AND RESULTS: Between January 1987 and January 1999, 592 patients underwent echocardiography after Fontan operation and 52 (8.8%) had intracardiac thrombus. Median age at Fontan operation was 1.9 years (range 0.8 to 35.1). Freedom from thrombus was 92%, 90%, 84% and 82% at 1, 3, 8, and 10 years after Fontan operation, respectively. There was no difference in freedom from thrombus, based on type of operation (atriopulmonary vs. lateral tunnel) or presence of fenestration. Thrombus was detected in the systemic venous atrium in 26 (48%), in the pulmonary venous atrium in 22 (44%), in both atria in 1 (2%), in the hypoplastic left ventricular cavity in 2 (8%), and in the ligated pulmonary artery stump in 1 (2%). CONCLUSIONS: Thrombus formation occurs with equal frequency in all types of modifications and is seen in the pulmonary, as well as the systemic venous atria. Our study suggests that thrombus formation is inherent to the physiology after Fontan operation and is not related to the type of modification performed.
Adolescent↗
Cerebral oxygen saturation before congenital heart surgery.
BACKGROUND: In congenital heart disease (CHD), neurologic abnormalities suggestive of hypoxia-ischemia are often apparent before cardiac surgery. To evaluate preoperative cerebral oxygenation, this study determined cerebral O2 saturation (ScO2) in CHD and healthy children. METHODS: Ninety-one CHD and 19 healthy children aged less than 7 years were studied before surgical or radiologic procedures. Arterial saturation (SaO2) and ScO2 were measured by pulse-oximetry and near infrared cerebral oximetry. Cerebral O2 extraction (CEO2) was calculated (SaO2-ScO2). SaO2, ScO2, and CEO2 were compared among diagnoses. Multivariable regression was performed between ScO2 and clinical variables. RESULTS: In healthy subjects, ScO2 (68%+/-10%) and CEO2 (30%+/-11%) were similar to patients with ventricular septal defect, aortic coarctation, and single ventricle after Fontan operation. ScO2 was significantly decreased in patients with patent ductus arteriosus (53%+/-8%), tetralogy of Fallot (57%+/-12%), hypoplastic left heart syndrome (46%+/-8%), pulmonary atresia (38%+/-6%), and single ventricle after aortopulmonary shunt (50%+/-7%), or bidirectional Glenn operation (43%+/-6%). CEO2 was significantly different only in patent ductus arteriosus (46%+/-8%) and hypoplastic left heart syndrome (38%+/-12%). In multivariable regression, only SaO2 was related to ScO2 (R = 0.63, p < 0.001). CONCLUSIONS: Cerebral oxygenation in CHD varies with anatomy and arterial saturation, and in some patients, it is very low compared with healthy subjects.
Brain Ischemia↗
Atrioventricular valve replacement in patients with a single ventricle.
BACKGROUND: Significant atrioventricular valve (AVV) insufficiency has been associated with increased mortality and morbidity in patients with single ventricle. Although many patients can be managed with valvuloplasty alone, some patients require AVV replacement. The optimal timing, outcome, and risk factors for AVV replacement in this population have not been described. METHODS: We retrospectively reviewed our experience with AVV replacement in patients with single ventricle from January 1984 to August 2000. Outcome variables included mortality and valve-related complications. RESULTS: Seventeen patients required AVV replacement. Prosthetic valve types included: St. Jude's valve in 14, Bjork-Shiley in 1, Hall-Kaster in 1, and Carpentier-Edwards in 1. Valve size ranged from 17 to 33 mm, Median age at valve replacement was 3.0 years (range 7 days to 17.3 years). Of the 16 subjects with normal atrioventricular conduction preoperatively, 7 (44%) developed postoperative complete heart block. Hospital mortality was 29%. Hospital mortality decreased significantly from 56% in 1984 to 1993 to no deaths from 1994 to 2000 (p = 0.03). Younger age (less than 2 years) at operation was also a risk factor for hospital mortality (p = 0.03). There were four late deaths in this series and 1 patient underwent heart transplantation. Of the surviving patients, none has required replacement of the prosthetic valve. No patients have had cerebrovascular accident subsequent to AVV replacement. Functional status is New York Heart Association functional class I in 5, class II in 1, and Class III in 1. CONCLUSIONS: Atrioventricular valve replacement can be performed in patients with single ventricle with acceptable morbidity and mortality. The development of postoperative complete heart block is common. Survival after AVV replacement has improved in recent years, and intervention before patients develop ventricular dysfunction and atrial arrhythmias may further improve outcome.
Adolescent↗
Atrioventricular valve regurgitation in patients with single ventricle: impact of the bidirectional cavopulmonary anastomosis.
BACKGROUND: It is not known whether atrioventricular valve regurgitation (AVVR) can improve in some patients after the bidirectional cavopulmonary anastomosis (BCPA) without undertaking concomitant valvuloplasty. METHODS: We retrospectively reviewed our experience with patients who underwent the BCPA between June 1989 and June 2000 with specific attention to those patients with moderate or severe AVVR. The degree of AVVR was determined by color Doppler echocardiography. During the study period there were 576 patients who underwent BCPA at a median age of 6.8 months (range, 1.3 months to 4.8 years). The majority of subjects (66%) had a tricuspid valve as the systemic atrioventricular valve. Significant (moderate or severe) AVVR before BCPA was noted in 36 of 576 patients (6%) and was significantly more prevalent (8 of 52 patients, 15%; p = 0.01) in patients with a common atrioventricular canal. RESULTS: Of the 36 subjects with moderate or severe AVVR, 8 had interventions (6 valvuloplasty, 2 aortic arch dilation) at BCPA. There was 1 hospital death (2.8%). Of the remaining 27 survivors who did not undergo additional interventions at BCPA, improvement in AVVR (mild or none) was noted in 6 (22%) at intermediate follow-up (median, 2 years; range, 0.5 to 3.1 years). The presence of significant AVVR before BCPA was not significantly associated with hospital survival or intermediate-term freedom from death or transplantation. CONCLUSIONS: We conclude that AVVR improves in some patients after BCPA and that valvuloplasty is not justified in all patients with moderate preoperative AVVR.
Anastomosis, Surgical↗
Cardiac surgery in the low-birth weight neonate. New approaches.
The LBW infant with congenital heart defects is likely to be at a higher risk for postoperative death and complications than their normal-sized counterparts because of the effects of prematurity and other organ system dysfunction. Current experience, however, does not suggest that waiting to obtain an arbitrary weight before surgery significantly improves outcome. Survival for low-weight infants undergoing complex repairs, such as those for interruption of the aortic arch and HLHS, is decreased compared with larger babies having the same operation at the same institution; however, the absolute survival rates are still quite acceptable. Weight alone should not be considered a contraindication to surgery for complex CHD. A careful investigation for the cause of the LBW must be undertaken (e.g., prematurity, infection, multiple congenital anomalies, and genetic abnormalities) and appropriate therapies initiated. The medical and surgical teams should collaborate to determine the optimal timing and type of surgery, with body weight being only of minor importance.
Heart Defects, Congenital↗
Outcome following tricuspid valve detachment for ventricular septal defects closure.
OBJECTIVES: Detachment of the septal leaflet of the tricuspid valve from the annulus (TVD) has been used to improve visualization of ventricular septal defects (VSDs), but may be associated with increased operative time, heart block, and the development of tricuspid regurgitation (TR). METHODS: Patients undergoing VSD closure between 1/1/96 and 31/12/99 were retrospectively reviewed. Follow-up was obtained from the patients' cardiologists. RESULTS: Transatrial VSD closure was performed in 172 patients with TVD in 36 (21%) at the surgeon's discretion. The leaflet incision was repaired with a separate suture (22) or with the VSD patch suture (14). Additional procedures including arch augmentation, closure of atrial septal defects, and closure of additional VSDs were performed in 93 (68%) non-TVD patients and 20 (56%) TVD patients. The median age was 6.2 months (range 1 day to 46 years) and the median weight was 5.9 kg (range 1.5-71.5 kg). Cardiopulmonary bypass (CPB) time was 64+/-24 min and cross-clamp time was 34+/-16 min. One hospital death occurred in an infant with tracheal stenosis. No child in either group developed complete heart block. The median duration of postoperative stay was 4 days (range 2-49 days). There were no differences in CPB time, cross-clamp time or postoperative stay between the TVD and non-TVD groups (P>0.1 for all). At a mean follow-up of 17+/-15 months, there have been two late deaths unrelated to cardiac disease. No child in the TVD group required reoperation for residual VSD, compared to three in the non-TVD group. No child in the TVD group has greater than mild TR, but six in the non-TVD group have greater than mild TR. No child in either group has undergone reoperation for TR. CONCLUSIONS: TVD is a safe, effective technique to improve visualization of VSD and is not associated with heart block, increased operative time, or TR. TVD may result in improved preservation of tricuspid valve architecture and decrease the incidence of significant postoperative TR.
Adolescent↗
Airway growth after pediatric lung transplantation.
BACKGROUND: Lung transplantation (LT) has been successfully offered to pediatric patients. Very little is known about the growth of the transplanted lung, especially in the infant population. Computerized tomography (CT) scanning is a simple method for studying pediatric patients who have undergone LT. We evaluated the use of CT scans to assess airway growth after pediatric LT, compare airway diameter indexed to somatic growth between LT patients and normals, and compare the growth of pre-anastomotic and post-anastomotic airways indexed to somatic growth in pediatric LT patients. METHODS: We reviewed CT scans on all pediatric patients who underwent primary LT before their fifteenth birthday between January 1995 and September 1998. Uniform measurements of diameter were made in pre-anastomotic (trachea, and proximal right and left bronchi) and post-anastomotic (distal right and left bronchi) sites. These measurements were then correlated with height and compared to previously published normal values. RESULTS: Of the 16 patients who underwent LT during the study period, 11 had at least 2 sequential CT scans (LT age 3 months to 14 years, median 2 years). Thirty-one CT scans were reviewed. Inter-observer variability was within 1 standard deviation (2 mm) in 93% of the measurements and inter-observer reliability was 0.91 by analysis of variance. Tracheal transverse diameter plotted against body height (slope 0.0072, correlation coefficient 0.88) was virtually identical to previously published norms. A similar relationship between airway diameter and height was observed in pre-anastomotic and post-anastomotic segments. CONCLUSION: CT scanning is a reliable method for assessing airway growth in pediatric LT recipients. Tracheal growth in pediatric LT recipients is similar to that of normal children. Post-anastomotic large airways grow similarly to native, pre-anastomotic airways.
Anastomosis, Surgical↗
Improved early results with cavopulmonary connections.
BACKGROUND: We describe the recent results in a large cohort of patients with functionally single ventricle who underwent various modifications of cavopulmonary connections. METHODS: Using the database at our institution, we identified all children who underwent cavopulmonary connection operations between June 1995 and June 1997. Demographic data, surgical history, and perioperative course were reviewed. RESULTS: We performed 130 consecutive operations in 113 patients. The procedures included superior cavopulmonary connections in the form of the HemiFontan procedure in 45 instances, and bidirectional Glenn procedures in 11, and bilateral superior cavopulmonary connections in 7. The median age of these patients was 7.0 months. We completed Fontan operations using a fenestrated lateral tunnel on 47 occasions, and using an extracardiac conduit 9 times, 5 of which were fenestrated. A lateral tunnel without fenestration was constructed in one patient. The median age for these procedures was 19.5 months. In the remaining 10 instances, we revised Fontan procedures at a median age of 8 years. Diagnoses included hypoplastic left heart syndrome in 43 patients, double outlet right ventricle in 22, heterotaxy in 13, tricuspid atresia in 13, and a miscellaneous group accounting for the other 22. One death (0.7%) occurred within 30 days of surgery. Clinical seizures occurred in 7 children (5.3%), 6 had no residual neurologic deficits. Atrial pacing was needed in 14 children (10.7%) because of transient junctional rhythm, and 2 received treatment for supraventricular tachycardia. Pleural effusions were diagnosed radiographically after 31 of 130 (24%) procedures. Diuretic therapy resolved the effusion in 21 of these, with only 6 children requiring thoracostomy catheter drainage, and 4 undergoing thoracentesis alone. The median length of stay on the intensive care unit was 2 days, with a range from 1 to 30 days, and median stay in hospital was 6 days, with a range from 3 to 58 days. CONCLUSION: Mortality and perioperative morbidity after cavopulmonary connections have decreased dramatically in the current era. The long-term results of staged reconstruction for functionally single ventricle, nonetheless, await ongoing study.
Cardiac Surgical Procedures↗
Sarcoma of the mitral valve causing coronary arterial occlusion in children.
Primary tumors of the cardiac valves are rare. One of the most common reasons that left-sided cardiac tumors come to clinical attention is embolization to the systemic circulation. We present two children who suffered left coronary arterial occlusion due to embolization of a sarcoma of the mitral valve. A 6-year-old female who had been admitted to the hospital after cerebrovascular embolization of a fragment of sarcoma of the mitral valve experienced sudden cardiovascular collapse due to occlusion of the left coronary artery. She was placed on extracorporeal membrane oxygenation, and underwent coronary embolectomy and resection of the tumor from the mitral valve and its tendinous cords. Left ventricular function did not improve, and she underwent orthotopic heart transplantation. On follow-up 32 months after transplant, the patient is well, with no evidence of recurrence of or metastasis from the tumor. The tumor arose from the leaflets and tendinous cords of the mitral valve, and was composed grossly of multiple white nodules. Histopathologic evaluation disclosed fragments composed predominantly of peripheral spindle cells in an extensive fibromyxoid stroma. The mildly pleomorphic cells of the tumor gradually blended with adjacent pieces of the mitral valvar leaflet and tendinous cords. Immunohistochemical studies revealed strong staining for vimentin, smooth muscle actin, muscle specific actin, and myoglobin, suggesting myogenic differentiation. The other patient was a 2 1/2-year-old female who died suddenly at home. Grossly and histologically, the tumor was essentially identical to the first case, and there was a 3 cm string-like extension passing into the orifice of the left coronary artery. To put the cases in context, we compare them with other descriptions of this rare type of tumor.
Child↗
Arrhythmias after pediatric lung transplantation.
Atrial arrhythmias have been reported after congenital heart surgery involving extensive atrial suture lines. Experimental studies involving bilateral lung transplantation (Tx) suggest that the left atrial suture lines predispose to atrial flutter. The overall incidence and type of arrhythmias after pediatric lung Tx have not previously been described and therefore the purpose of this study was to prospectively screen and describe arrhythmias in a subset of our lung transplant population. Over a 1-yr study period, all recipients of bilateral lung Tx were admitted to a full-disclosure telemetry unit. Single-lead electrocardiograms were recorded continuously and reviewed daily via a beat-by-beat analysis. A total of 314 patient days (range 9-93, median 43 days) were recorded from seven patients. The incidence of arrhythmias observed per total patient days included junctional escape rhythm (4.8%), non-sustained ventricular tachycardia (4.1%), accelerated junctional (2.5%), sinus bradycardia (2.2%), non-sustained supraventricular tachycardia (1.3%), ectopic atrial tachycardia (1.0%), sustained ventricular tachycardia (0.3%), junctional ectopic tachycardia (0.3%), and second degree heart block (0.3%). No patient had sustained supraventricular tachycardia, atrial flutter, atrial fibrillation, or complete heart block. Arrhythmias were treated in two patients. During the follow-up period, one patient received amiodarone for ventricular tachycardia (which was also noted and treated prior to transplant). We conclude that among pediatric lung transplant recipients admitted for their transplant surgery, arrhythmia is uncommon and rarely requires therapy.
Adolescent↗
Metachronous Epstein-Barr virus-related smooth muscle tumors in a child after heart transplantation: case report and review of the literature.
Soft tissue tumors are uncommon manifestations of Epstein-Barr virus (EBV) infection in patients who have had transplants. The authors report 2 metachronous EBV-containing smooth muscle tumors in a child who had a heart transplant, and review the literature on posttransplant soft tissue tumors.
Biopsy, Needle↗
Atrial pacing: an alternative treatment for protein-losing enteropathy after the Fontan operation.
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Cardiac Pacing, Artificial↗
Aortic homograft interposition for management of complete tracheal anastomotic disruption after heart-lung transplantation.
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Adult↗