Altered breathing mechanics and ventilatory response during exercise in children born extremely preterm.

BACKGROUND: Extreme preterm birth confers risk of long-term impairments in lung function and exercise capacity. There are limited data on the factors contributing to exercise limitation following extreme preterm birth. This study examined respiratory mechanics and ventilatory response during exercise in a large cohort of children born extremely preterm (EP). METHODS: This cohort study included children 8-12 years of age who were born EP (≤28 weeks gestation) between 1997 and 2004 and treated in a large regionalised neonatal intensive care unit in western Canada. EP children were divided into no/mild bronchopulmonary dysplasia (BPD) (ie, supplementary oxygen or ventilation ceased before 36 weeks gestational age; n=53) and moderate/severe BPD (ie, continued supplementary oxygen or ventilation at 36 weeks gestational age; n=50). Age-matched control children (n=65) were born at full term. All children attempted lung function and cardiopulmonary exercise testing measurements. RESULTS: Compared with control children, EP children had lower airway flows and diffusion capacity but preserved total lung capacity. Children with moderate/severe BPD had evidence of gas trapping relative to other groups. The mean difference in exercise capacity (as measured by oxygen uptake (VO2)% predicted) in children with moderate/severe BPD was -18±5% and -14±5.0% below children with no/mild BPD and control children, respectively. Children with moderate/severe BPD demonstrated a potentiated ventilatory response and greater prevalence of expiratory flow limitation during exercise compared with other groups. Resting lung function did not correlate with exercise capacity. CONCLUSIONS: Expiratory flow limitation and an exaggerated ventilatory response contribute to respiratory limitation to exercise in children born EP with moderate/severe BPD.

Successful Semi-Ambulatory Veno-Arterial Extracorporeal Membrane Oxygenation Bridge to Heart-Lung Transplantation in a Very Small Child.

Lung transplantation (LTx) may be denied for children on extracorporeal membrane oxygenation (ECMO) due to high risk of cerebral hemorrhage. Rarely has successful LTx been reported in children over 10 years of age receiving awake or ambulatory veno-venous ECMO. LTx following support with ambulatory veno-arterial ECMO (VA ECMO) in children has never been reported to our knowledge. We present the case of a 4-year-old, 12-kg child with heritable pulmonary artery hypertension and refractory right ventricular failure. She was successfully bridged to heart-lung transplantation (HLTx) using ambulatory VA ECMO. Initial resuscitation with standard VA ECMO was converted to an ambulatory circuit using Berlin heart cannulae. She was extubated and ambulating around her bed while on VA ECMO for 40 days. She received an HLTx from an oversized marginal lung donor. Despite a cardiac arrest and Grade 3 primary graft dysfunction, she made a full recovery without neurological deficits. She achieved 104% force expiratory volume in 1 s 33 months post-HLTx. Ambulatory VA ECMO may be a useful strategy to bridge very young children to LTx or HLTx. Patient tailored ECMO cannulation, minimization of hemorrhage, and thrombosis risks while on ECMO contributed to a successful HLTx in our patient.

Prenatal diagnosis of primary pulmonary hypoplasia in fraternal twins.

Primary pulmonary hypoplasia is a rare, usually lethal, condition presenting only after birth without other congenital abnormalities. We describe the first case of fraternal twins diagnosed prenatally with primary pulmonary hypoplasia. Both had diffuse hypoplasia of the pulmonary arteries initially identified by fetal echocardiography and confirmed at autopsy following termination. These cases permit examination of the histopathology of this disease in the fetal stage of development.

Unroofed coronary sinus and coronary sinus orifice atresia. Implications for management of complex congenital heart disease.

OBJECTIVES: The aim of this study was to assess the morphology of the coronary sinus, its drainage and associated cardiac malformations when there is either complete unroofing of the coronary sinus or atresia of its connection to the right atrium. BACKGROUND: As more children with complex cardiac anomalies are accepted for primary surgical repair or palliation with cavopulmonary anastomoses, a knowledge of coronary sinus and systemic venous anomalies is important if coronary venous return is to be preserved and residual shunts avoided. METHODS: Twenty-six heart-lung specimens without a coronary sinus draining to the right atrium were identified from the Leiden collection of congenital heart malformations. These were classified into specimens with an unroofed coronary sinus and those with atresia of the coronary sinus orifice. Attention was paid to the associated cardiac malformations. RESULTS: In 14 (54%; confidence limits [CL] 35%, 73%) of 26 specimens, there was an unroofed coronary sinus, associated with persistence of the left superior caval vein. An inferoposterior location of an atrial septal defect was detected in 2 (14%; CL -4%, 33%) of 14. Atrial appendage anomalies were seen in 13 (93%; CL 79%, 106%) of 14 specimens, exemplified by both right and left isomerism. These were frequently associated with an atrioventricular septal defect (12 [86%; CL 67%, 104%] of 14). An atretic coronary sinus orifice was seen in 12 (46%; CL 27%, 65%) of 26. Atrial appendage anomalies (2 [17%; CL -4%, 38%] of 12) were rare in these cases. The drainage was then by way of a left superior caval vein or, in its absence, a coronary sinus to left atrial window. Ventricular hypoplasia was seen in both categories of coronary sinus abnormalities. Important ventricular hypoplasia was seen in 12 cases (46%; CL 27%, 65%). CONCLUSIONS: These findings emphasize the need to study coronary sinus drainage before procedures such as ligation or transcatheter coil embolization of a left superior caval vein, venous redirection or closure of a dorsal atrial septal defect are contemplated. These procedures might inadvertently lead to impairment of coronary venous return or persistence of an intracardiac shunt.

Combined effects of nitric oxide and oxygen during acute pulmonary vasodilator testing.

OBJECTIVES: We compared the ability of inhaled nitric oxide (NO), oxygen (O2) and nitric oxide in oxygen (NO+O2) to identify reactive pulmonary vasculature in pulmonary hypertensive patients during acute vasodilator testing at cardiac catheterization. BACKGROUND: In patients with pulmonary hypertension, decisions regarding suitability for corrective surgery, transplantation and assessment of long-term prognosis are based on results obtained during acute pulmonary vasodilator testing. METHODS: In group 1, 46 patients had hemodynamic measurements in room air (RA), 100% O2, return to RA and NO (80 parts per million [ppm] in RA). In group 2, 25 additional patients were studied in RA, 100% O2 and 80 ppm NO in oxygen (NO+O2). RESULTS: In group 1, O2 decreased pulmonary vascular resistance (PVR) (mean+/-SEM) from 17.2+/-2.1 U.m2 to 11.1+/-1.5 U.m2 (p < 0.05). Nitric oxide caused a comparable decrease from 17.8+/-2.2 U.m2 to 11.7+/-1.7 U.m2 (p < 0.05). In group 2, PVR decreased from 20.1+/-2.6 U.m2 to 14.3+/-1.9 U.m2 in O2 (p < 0.05) and further to 10.5+/-1.7 U.m2 in NO+O2 (p < 0.05). A response of 20% or more reduction in PVR was seen in 22/25 patients with NO+O2 compared with 16/25 in O2 alone (p = 0.01). CONCLUSIONS: Inhaled NO and O2 produced a similar degree of selective pulmonary vasodilation. Our data suggest that combination testing with NO + O2 provides additional pulmonary vasodilation in patients with a reactive pulmonary vascular bed in a selective, safe and expeditious fashion during cardiac catheterization. The combination of NO+O2 identifies patients with significant pulmonary vasoreactivity who might not be recognized if O2 or NO were used separately.

Clinical course and hemodynamic observations after supraannular mitral valve replacement in infants and children.

OBJECTIVES: We report the clinical course and unique hemodynamic findings after placement of a supraannular mitral valve prosthesis. BACKGROUND: Children with symptomatic mitral valve disease whose annulus is too small for the smallest prosthesis are difficult to manage. One option is valve replacement with a prosthesis positioned entirely within the left atrium (LA). METHODS: We reviewed 17 patients (median age 10 months) with symptomatic mitral valve disease who underwent placement of a supraannular valve prosthesis between 1980 and 1994. RESULTS: The actuarial survival rates were 88% at 1 month and 71%, 62% and 53% at 1, 2 and 10 years, respectively. Preoperative hemodynamic data (mean +/- SD)) compared with those after placement of the supraannular mitral prosthesis were as follows: "a" wave to left ventricular end-diastolic pressure gradient 17 +/- 5 versus 4 +/- 4 mm Hg (p = 0.003), mean LA pressure 25 +/- 6 versus 20 +/- 6 mm Hg (p = 0.07), "a" wave 30 +/- 6 versus 19 +/- 5 mm Hg (p = 0.006), "v" wave 28 +/- 5 versus 30 +/- 9 mm Hg (p = 0.31), mean pulmonary artery pressure 54 +/- 19 versus 42 +/- 15 mm Hg (p = 0.07) and left ventricular end-diastolic pressure 14 +/- 5 versus 16 +/- 4 mm Hg (p = 0.12). CONCLUSIONS: Supraannular mitral valve replacement provides relief of mitral stenosis or mitral regurgitation. However, LA to left ventricular early diastolic gradients with large atrial "v" waves contribute to elevated mean LA pressures in the absence of prosthetic valve obstruction or regurgitation. As a result of this unexpected finding, associated left heart obstructive lesions and pulmonary and left ventricular end-diastolic hypertension, the outlook remains poor.

Balloon angioplasty of recurrent coarctation: a 12-year review.

OBJECTIVES: This study was undertaken to investigate the long-term outcome of balloon angioplasty for recurrent coarctation of the aorta in a large series of patients. BACKGROUND: Balloon angioplasty has become the standard treatment for residual or recurrent aortic coarctation. Despite the widespread use of this treatment modality, there are few data outlining the long-term outcome of a large patient cohort. METHODS: Clinical, echocardiographic, hemodynamic and angiographic data on 90 consecutive patients who underwent balloon angioplasty between January 1984 and January 1996 were reviewed. RESULTS: Mean systolic pressure gradients were reduced from 31 +/- 21 to 8 +/- 9 mm Hg after dilation (p = 0.0001). The mean diameter of the stenotic site, measured in the frontal and lateral views, increased by 38% and 35%, respectively (p = 0.001). Neurologic events occurred in two patients, with one death. An aortic tear occurred in one patient, requiring surgical intervention. Optimal results were defined as a postprocedure gradient < 20 mm Hg and were obtained acutely in 88% of patients. At long-term follow-up (12 years), 53 (72%) of 74 patients with an early optimal result remained free from reintervention. Transverse arch hypoplasia, defined as an arch dimension < 2 SD below the mean for age, was the primary predictor of the need for reintervention. CONCLUSIONS: Although the majority of patients undergoing percutaneous balloon angioplasty for recoarctation of the aorta will achieve long-term benefit, the need for further surgical intervention in those with transverse arch hypoplasia remains high.

Inhaled nitric oxide and hemodynamic evaluation of patients with pulmonary hypertension before transplantation.

OBJECTIVES: We investigated the effect of inhaled nitric oxide and infused acetylcholine in patients with pulmonary hypertension undergoing cardiac catheterization before cardiopulmonary transplantation. BACKGROUND: The fate of patients under consideration for transplantation of the heart or lungs, or both, is influenced by the evaluation of their pulmonary vascular reactivity. METHODS: We evaluated 11 patients who were classified into two groups on the basis of mean left atrial pressure > 15 mm Hg (group I, n = 6) or < or = 15 mm Hg (group II, n = 5). All patients inhaled nitric oxide at 80 ppm. This was preceded by an infusion of 10(-6) mol/liter of acetylcholine in seven consecutive patients (n = 3 in group I, n = 4 in group II). RESULTS: In group I, inhaled nitric oxide decreased pulmonary artery pressure from (mean +/- SE) 71 +/- 13 to 59 +/- 10 mm Hg (p < 0.05), pulmonary vascular resistance from 14.9 +/- 3.8 to 7.6 +/- 1.7 Um2 (p < 0.05) and intrapulmonary shunt fraction from 17.8 +/- 3.6% to 12.7 +/- 2.1% (p < 0.05). Left atrial pressure tended to increase from 27 +/- 4 to 32 +/- 5 mm Hg (p = 0.07). In group II pulmonary vascular resistance decreased in response to nitric oxide from 36.4 +/- 9.0 to 31.1 +/- 7.9 Um2 (p < 0.05). Cardiac index, systemic pressure and resistance did not change in either group. Seven patients who received acetylcholine had no significant alteration in pulmonary hemodynamic variables. CONCLUSIONS: These preliminary observations suggest that nitric oxide is a potent pulmonary vasodilator with minimal systemic effects. It may be useful in discriminating patients needing combined heart and lung transplantation from those requiring exchange of the heart alone.

Mortality in potential arterial switch candidates with transposition of the great arteries.

OBJECTIVES: We reviewed the factors contributing to or causing death before surgery in neonates with d-transposition of the great arteries (TGA) despite anatomy suitable for the arterial switch operation (ASO) to develop strategies to minimize preoperative attrition. BACKGROUND: Currently the ASO for neonates with TGA carries a low operative mortality. However, there is a paucity of information regarding the patients who die before the ASO. Strategies to ensure survival to operation are of importance to improve overall outcome. METHODS: We reviewed all neonates with TGA and patent forearm ovale (PFO) < or = 2 mm, a birthweight <2 kg, or who died before surgery, between 1988 and 1996. RESULTS: We identified 12 out of 295 neonates with TGA (4.1%) with anatomy suitable for the ASO who died prior to surgery. All had TGA/intact ventricular septum (IVS) and presented with a severely restrictive PFO. In 11 of 12 cases the cause of death was attributed to the sequelae of profound hypoxemia from inadequate mixing. Contributing factors were prematurity, 41.7%; severe respiratory distress syndrome, 25%; and persistent pulmonary hypertension of the newborn (PPHN), 16.7%. All patients received prostaglandin E1 (PGE1) infusion. Urgent balloon atrial sepstostomy (BAS) was performed in 66.7% with improved oxygenation. No cases were diagnosed prenatally. In contrast, all patients with a PFO < or = 2 mm who survived to ASO had a significantly better response to PGE1 infusion (p=0.03) than nonsurvivors. The ASO was accomplished without mortality in four of nine with a weight <2 kg. CONCLUSIONS: Of those neonates admitted with TGA, 4.1% died before surgery. Eleven of 12 (3.7%) died due to consequences of inadequate interatrial mixing despite PGE1 infusion. Earlier diagnosis and BAS are critically important in determining survival. Early ASO may improve survival in patients weighing <2 kg. Prenatal diagnosis with delivery in a high-risk obstetrical unit with facilities for immediate BAS and supportive therapy for pulmonary hypertension and ventricular failure may be necessary to salvage this group of patients.

Inhaled nitric oxide in children with pulmonary hypertension and congenital mitral stenosis.

We demonstrated selective pulmonary vasodilation in patients with congenital mitral stenosis with inhaled NO, and used NO as prolonged therapy to stabilize the postintervention course of these children. The pulmonary vasoreactivity was greater than that previously reported in adults and may be due to particular sensitivity of pulmonary veins to inhaled NO when pulmonary venous hypertension has been present since birth.

Transverse limb defects associated with aorto-pulmonary vascular abnormalities: vascular disruption sequence or atypical presentation of Adams-Oliver syndrome?

We report a patient with terminal transverse limb defects associated with persistent primitive aorto-pulmonary vascular connections leading to supra-systemic pulmonary artery pressure. It is likely that this patient represents a vascular disruption sequence or as an alternative a form of Adams-Oliver syndrome. These assumptions are based only on the association of vascular abnormalities as an emerging and apparently important association with transverse limb defects despite the absence of aplasia cutis congenita commonly associated with Adams-Oliver syndrome.

Increased plasma levels of endothelin-1 after cardiopulmonary bypass in patients with pulmonary hypertension and congenital heart disease.

The plasma level of the potent vasoconstrictor endothelin-1 was measured in children who underwent cardiac operations. Forty-five patients were divided into two groups, those with a high pulmonary blood flow (HF group; n = 23) and those with a normal or low flow (NF group; n = 22). Seven blood samples were taken: immediately before cardiopulmonary bypass, immediately after removing the aortic cross-clamps, immediately after discontinuing bypass, and at 20 minutes and 3, 6, and 24 hours after termination of bypass. The plasma levels of endothelin-1 were similar in both groups before bypass. From the time the aortic crossclamps were removed, the plasma endothelin-1 levels in both groups increased significantly, to reach a peak level at 3 to 6 hours. The increase was significantly greater in the HF than in the NF group, and the maximum values in the two groups were 12.6 +/- 1.1 and 9.6 +/- 0.8 fmol/ml, respectively (mean +/- standard error of the mean, p < 0.05). The value 20 minutes after bypass showed a positive correlation with the mean pulmonary arterial pressure measured at the preoperative cardiac catheterization study (r = 0.41, p < 0.05). In addition, a significant positive correlation was obtained between endothelin-1 3 hours after bypass and the maximum pulmonary/systemic arterial pressure ratio during the first 12 hours after operation (r = 0.86, p < 0.05). These results suggest that cardiopulmonary bypass is associated with an immediate postoperative increase in circulating endothelin and that patients who had a high pulmonary blood flow before the operation are particularly vulnerable, bypass having a more injurious effect on a lung with preexisting endothelial dysfunction. A high level of circulating endothelin may predispose to pulmonary vascular lability and pulmonary hypertensive crises in the postoperative period.

Inhaled nitric oxide differentiates pulmonary vasospasm from vascular obstruction after surgery for congenital heart disease.

OBJECTIVE: To evaluate whether a trial of inhaled nitric oxide (NO) differentiates reversible pulmonary vasoconstriction from fixed anatomic obstruction to pulmonary blood flow after surgery for congenital heart disease in patients at risk for pulmonary hypertension. DESIGN: Prospective cohort study. SETTING: Tertiary care children's hospital. PATIENTS: 15 neonate and infants with elevated pulmonary artery or right ventricular pressure or with clinical signs suggestive of high pulmonary vascular resistance in the early postoperative period following repair of congenital heart disease. INTERVENTION: 30-min trial of 40 ppm inhaled NO. RESULTS: 5 patients responded to inhaled NO, 2 patients were weaned from extracorporeal support with NO. Four were maintained on continuous inhaled NO for 3 to 5 days. All the responders survived. Ten patients did not respond to NO. An important anatomic obstruction was found with echocardiography or angiography in all 10 patients. Reintervention was performed in 6/10 (4 stent placement, 1 balloon angioplasty of pulmonary arteries and 1 revision of systemic to pulmonary shunt). Six of the nonresponders died. CONCLUSION: A trial of inhaled NO after cardiac surgery in neonates and infants may be useful to differentiate reversible pulmonary vasoconstriction from fixed anatomic obstruction and may provide useful information if temporary support with extracorporeal membrane oxygenation is considered. Failure to respond to inhaled NO should prompt further investigations to rule out a residual obstruction.

Rebound pulmonary hypertension after inhalation of nitric oxide.

BACKGROUND: We describe the hemodynamic response to initiation and withdrawal of inhaled nitric oxide (NO) in infants with pulmonary hypertension after surgical repair of total anomalous pulmonary venous connection. METHODS: Between January 1, 1992, and January 1, 1995, 20 patients underwent repair of total anomalous pulmonary venous connection. Nine patients had postoperative pulmonary hypertension and received a 15-minute trial of inhaled NO at 80 parts per million. Five of these patients received prolonged treatment with NO at 20 parts per million or less. RESULTS: Mean pulmonary artery pressure decreased from 35.6 +/- 2.4 to 23.7 +/- 2.0 mm Hg (mean +/- standard error of the mean) (p = 0.008), and pulmonary vascular resistance decreased from 11.5 +/- 2.0 to 6.4 +/- 1.0 U.m2 (p = 0.03). After prolonged treatment with NO, pulmonary artery pressure increased transiently in all patients when NO was discontinued. CONCLUSIONS: After operative repair of total anomalous pulmonary venous connection, inhaled NO selectively vasodilated all patients with pulmonary hypertension. Withdrawal of NO after prolonged inhalation was associated with transient rebound pulmonary hypertension that dissipated within 60 minutes. Appreciation of rebound pulmonary hypertension may have important implications for patients with pulmonary hypertensive disorders when interruption of NO inhalation is necessary or when withdrawal of NO is planned.

Truncal valve repair: initial experience in neonates.

BACKGROUND: The identification of moderate to severe preoperative truncal valve regurgitation has been synonymous with significant postoperative mortality after neonatal repair of truncus arteriosus. Spurned by the deficiencies of current truncal valve substitutes surgeons are once again reexamining the option of truncal valve reparative techniques. METHODS: From May 1996 until June 1997, 8 children underwent correction of truncus arteriosus. A retrospective analysis was conducted. RESULTS: There was one in-hospital death secondary to a postoperative massive coronary air embolism. Moderate-to-severe truncal valve regurgitation was identified clinically and confirmed with cardiac ultrasound in 3 neonates with a mean age of 7 days (range, 4 to 12 days) all with quadracusp truncal valves. Successful truncal valve repair was accomplished in 2 infants, with a third neonate requiring homograft replacement with coronary reimplantation for failure in achieving valvular competence after attempted valvuloplasty. Postoperative echocardiograms in those neonates who underwent truncal valve repair confirmed a functional "tricuspid" valve with only mild to mild-plus regurgitation. CONCLUSIONS: We give further credence to the hypothesis that primary neonatal truncal valve repair is feasible and may be successful in the avoidance and delay of serial truncal valve replacements using either mechanical or allograft substitutes.

Chylopericardium after cardiac operations in children.

BACKGROUND: Chylopericardium is a rare complication after operation for congenital heart disease. The incidence and clinical outcomes in a large cohort of surgical patients are unknown. METHODS: We retrospectively reviewed the clinical records spanning more than 12 years in a single institution of 16 children with chylopericardium after cardiac operation. RESULTS: We identified 16 patients with chylopericardium between 1985 and 1997. Chylopericardium was isolated in 7 patients. Twelve patients required pericardial drainage. Patients with isolated chylopericardium presented late and were treated initially as having postpericardiotomy syndrome. Three patients underwent thoracic duct ligation. There were two late deaths unrelated to the chylothorax. Associated diagnoses were internal jugular vein thrombosis and recurrent pulmonary vein obstruction (1 of 16 patients), an associated syndrome but not Turner or Noonan (10 of 16), superior cavopulmonary or total cavopulmonary anastomosis (7 of 16), atrioventricular septal defect repair (5 of 16), and repair of tetralogy of Fallot (2 of 16). CONCLUSIONS: Percutaneous drainage to relieve tamponade together with a low-fat or medium-chain triglyceride diet results in resolution in most cases of postoperative chylopericardium. If a pericardial effusion enlarges, fails to clear on aspirin therapy, or presents late after hospital discharge, diagnostic pericardial tap and a low-fat diet are indicated.

Precoronary stenosis after stage I palliation for hypoplastic left heart syndrome.

We report a patient with stenosis of the native ascending aorta after palliation of hypoplastic left heart syndrome and aortic atresia. We describe the approach to diagnosis, temporary support with extracorporeal membrane oxygenation, and successful surgical reintervention. Stenosis of the native ascending aorta is an important, potentially reversible cause of acute, early postoperative ventricular dysfunction.

Decreased exhaled nitric oxide may be a marker of cardiopulmonary bypass-induced injury.

BACKGROUND: Nitric oxide is an endothelium-derived vasodilator. Cardiopulmonary bypass may induce transient pulmonary endothelial dysfunction with decreased nitric oxide release that contributes to postoperative pulmonary hypertension and lung injury. Exhaled nitric oxide levels may reflect, in part, endogenous production from the pulmonary vascular endothelium. METHODS: We measured exhaled nitric oxide levels before and 30 minutes after cardiopulmonary bypass in 30 children with acyanotic congenital heart disease and left-to-right intracardiac shunts undergoing repair. RESULTS: Exhaled nitric oxide levels decreased by 27.6%+/-5.6% from 7+/-0.8 to 4.4+/-0.5 ppb (p < 0.05) 30 minutes after cardiopulmonary bypass despite a reduction in hemoglobin concentration. CONCLUSIONS: The decrease in exhaled nitric oxide levels suggests reduced nitric oxide synthesis as a result of pulmonary vascular endothelial or lung epithelial injury. This may explain the efficacy of inhaled nitric oxide in the treatment of postoperative pulmonary hypertension. Furthermore, strategies aimed at minimizing endothelial dysfunction and augmenting nitric oxide production during cardiopulmonary bypass may decrease the incidence of postoperative pulmonary hypertension. Exhaled nitric oxide levels may be useful to monitor both cardiopulmonary bypass-induced endothelial injury and the effect of strategies aimed at minimizing such injury.

Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes.

BACKGROUND: The historic outcome of the Fontan procedure in children with single ventricle and heterotaxy syndrome has been poor, and in the current era it has been incompletely described. METHODS: From January 1993 through April 2000, 30 patients (age range, 5.3 +/- 3.6 years) had total cavopulmonary connection for single ventricle and isomerism and heterotaxy syndrome. Right atrial isomerism and heterotaxy (n = 19) or left atrial isomerism and heterotaxy (n = 11) were associated with a morphologic right ventricle (n = 26), a common atrioventricular valve (n = 25), pulmonary atresia (n = 9) or stenosis (n = 17), anomalous pulmonary venous drainage (partial, n = 7; total, n = 11; obstructed, n = 4), and interrupted inferior vena cava (n = 13). A previous systemic to pulmonary artery shunt had been performed in 21 patients, and 3 patients had previous pulmonary artery banding. The Fontan procedure was staged in all but 3 patients at a median age of 12 months (range, 3 to 114 months). Before the Fontan procedure, atrial arrhythmia was present in 7 patients (23%). RESULTS: An extracardiac conduit was constructed in 22 patients (median diameter, 20 mm; range, 16 to 27 mm), and a lateral tunnel was constructed in 4 patients. Associated procedures included pulmonary artery augmentation (n = 17), repair of anomalous pulmonary venous drainage (n = 7), and repair of a regurgitant atrioventricular valve (n = 2). The mean cardiopulmonary bypass time was 134 +/- 57 minutes. Cardioplegic cardiac arrest was used in 12 patients for a mean duration of 62 +/- 28 minutes. There were four hospital deaths (13%), three that occurred in children having concomitant repair of anomalous pulmonary venous drainage (previously undetected in one). Thirteen children (41%) developed early postoperative atrial arrhythmias, 11 of the 13 children required temporary pacing. There was one late death. Follow-up (3.4 +/- 2.7 years) was available on 24 of 25 survivors. All but 2 patients were in sinus rhythm. CONCLUSIONS: Early and midterm outcomes after the Fontan procedure in this patient group improved and may be further ameliorated by detecting and repairing associated obstruction to pulmonary venous flow and performing atrioventricular valvuloplasty before the Fontan procedure.

Inhaled nitric oxide in the treatment of postoperative graft dysfunction after lung transplantation.

Pulmonary hypertension and transient graft dysfunction may complicate the postoperative course of patients undergoing lung transplantation. We report the acute effect of inhaled nitric oxide (80 ppm) on hemodynamics and gas exchange in 6 patients (median age, 14 years; range, 5 to 21 years) after lung transplantation as well as the effect of extended treatment over 40 to 69 hours in 2 patients. In 5 patients with pulmonary hypertension nitric oxide lowered mean pulmonary artery pressure (from 38.4 +/- 1.6 to 29.4 +/- 3.1 mm Hg; p < 0.05), pulmonary vascular resistance index (from 9.3 +/- 1.4 to 6.4 +/- 1.3 Um2; p < 0.05), and intrapulmonary shunt fraction (from 28.6% +/- 8.3% to 21.0% +/- 5.7%; p < 0.05). There was a 28.4% +/- 7.2% reduction in transpulmonary pressure gradient with only minor accompanying effects on the systemic circulation. Mean arterial pressure decreased only 2.7% +/- 5% (from 76.4 +/- 2.2 to 74 +/- 2.3 mm Hg; p = not significant), and systemic vascular resistance index by 4.2% +/- 9.7% (from 21.7 +/- 3.1 to 20.6 +/- 3.6 Um2; p = not significant). Cardiac index was unchanged (from 3.5 +/- 0.8 to 3.6 +/- 0.7 L.min-1.m-2; p = not significant). Nitric oxide caused a sustained improvement in oxygenation and pulmonary artery pressure during extended therapy at doses of 10 ppm. There were no major side effects. However, transient methemoglobinemia (9%) developed in 1 patient after 10 hours of nitric oxide treatment. Nitric oxide may be useful in the treatment of pulmonary hypertension and the impaired gas exchange that occurs after lung transplantation.