Thrombolysis of Prosthetic Tricuspid Valve Thrombosis with Human Recombinant Tissue Plasminogen Activator in an Adolescent

Prosthetic heart valve thrombosis is associated with a high mortality. Traditionally, thrombectomy or valve replacement is performed. Thrombolysis offers a promising alternative to surgery. Usually, streptokinase and urokinase are the preferred agents for thrombolysis; however, human recombinant tissue plasminogen activator (rt-PA) is increasingly used. Thrombosis of prosthetic valves in children and adolescents is rare and experience of thrombolysis for obstructed valves is limited. We report the successful lysis of a thrombosed prosthetic tricuspid valve in an adolescent using rt-PA. The outcome of our patient supports the assumption that rt-PA represents an adequate therapeutic option for thrombolysis of obstructed prosthetic heart valves in children and adolescents.     

Fontan Patient with Plastic Bronchitis Treated Successfully Using Aerosolized Tissue Plasminogen Activator: A Case Report and Review of the Literature

Plastic bronchitis is an uncommon condition characterized by the production of large pale bronchial casts that obstruct the tracheobronchial tree. The cellular content, cohesiveness, and often rubber-like consistency distinguish bronchial casts from the usual mucus plugs found with such disease states as asthma. Plastic bronchitis can be found secondary to many conditions, and a simplified classification scheme organizes it into two groups: an inflammatory type consisting of casts with an eosinophilic inflammatory infiltrate and an acellular type with a predominance of fibrin distinguished by its relative lack of cellular infiltrate, its mucin predominance, and its appearance only in children with congenital cyanotic heart disease. This report describes a 5-year-old girl who experienced plastic bronchitis 3 months after a Fontan procedure for hypoplastic left heart syndrome that was treated successfully with aerosolized tissue plasminogen activator.     

Acute Pulmonary Embolism Resulting from Cardiac Tumors Associated with Tuberous Sclerosis

A case report of a neonate with cardiac tumors in association with tuberous sclerosis is presented. The rare complication of pulmonary embolism and its successful treatment by anticoagulation and thrombolytic therapy is described. The treatment of these cardiac tumors is conservative, because the tumors regress spontaneously when the diagnosis is made in infancy. However, embolic event may rarely occur as a complication of cardiac tumors. If the cardiac tumors are treated conservatively, it is important to pay close attention to the occurrence of pulmonary embolism during the follow-up period.     

Surgical Ligation of Anomalous Hepatic Vein in a Case of Heterotaxy Syndrome with Massive Intrahepatic Shunting After Modified Fontan Operation

In case of progressive cyanosis after modified Fontan procedure, intrahepatic shunting should be considered. Ligation of the hepatic vein is safe and effective in managing this condition, and it is important to ensure that the pressure difference between superior vena cava and the portal vein system remains minimal after clamping of the hepatic vein before ligation is completed to minimize the possibility of varix due to portal vein hypertension.     

Fenestrated Fontan Procedure: Evolution of Technique and Occurrence of Paradoxical Embolism

The Fenestrated Fontan procedure (FFP) has improved outcome in high risk patients. The technique is evolving, however, and complications are not fully known. Over a 3-year period 13 patients (mean age 35 ± 29 months) underwent an FFP in our institution. In the first two patients the fenestration had to be created because of high right atrial pressure and low cardiac output; in 11 patients the FFP was planned. In three patients the sutures for the adjustable fenestration were crossing the defect. In 10 patients, purse-string sutures were placed around but not across the defect. Because large fenestrations were created in 11 patients (8–12 mm) Glenn shunts were performed to improve arterial saturation. The postoperative course was relatively uneventful, with chest tubes being removed 1–8 days (mean 4 ± 3 days) postoperatively and the hospital stay ranging from 7 to 27 days (mean 14 ± 6 days). One patient had bleeding and another had a mediastinal abscess. The first patient died (7.6%) because of hemodynamic instability due to prolonged cardiopulmonary bypass from the creation and enlargement of the fenestration. One patient had a paradoxical cerebral embolism from clots that formed on the sutures crossing the fenestration. Because of this problem the remaining patients were placed on salicylates while awaiting closure of their fenestration. All 12 patients had their fenestrations closed, performed under local anesthesia in 9, at mediastinal abscess drainage in 1, and spontaneously in 2. We conclude that creation of large fenestrations in combination with Glenn shunts and the use of adjustable fenestrations are viable modifications of the FFP. The use of purse-string sutures around the fenestration and antiplatelet drugs can probably minimize the occurrence of paradoxical embolism.     

Acute Pulmonary Embolism in a Neonate: Precipitation During Cardiac Catheterization and Successful Treatment

This report illustrates the rare occurrence of a pulmonary embolus in a neonate during cardiac catheterization. The patient was a term newborn who underwent repair of obstructed infradiaphragmatic total anomalous pulmonary venous connection. Postoperative risk factors for pulmonary embolism included severe pulmonary hypertension unresponsive to nitric oxide therapy, an indwelling venous catheter, and young age. Successful management was achieved by initial mechanical fragmentation with streptokinase infusion and monitoring by serial lung perfusion scans.     

Responses of Plasma Norepinephrine and Heart Rate During Exercise in Patients After Fontan Operation and Patients with Residual Right Ventricular Outflow Tract Obstruction After Definitive Reconstruction

To determine the exercise responses of patients with congenital heart disease, 20 patients—5 who had undergone a right ventricular outflow tract reconstruction (group R; age, 15 ± 2 years), eight who had undergone a Fontan operation (group F; age, 13 ± 2 years), and seven who had a history of Kawasaki disease (group C; age, 15 ± 1 years)—performed a treadmill exercise test. Patients of group R had a significant residual right ventricular outflow obstruction. Oxygen uptake (VO₂), heart rate (HR), and plasma norepinephrine (NE) concentrations were measured at rest, during warm-up, at ventilatory threshold (VT), and at peak exercise. Exercise capacity was determined as a percentage of the predicted normal peak VO₂ (%pVO₂). The %pVO₂ for groups R and F was 65 ± 10 and 56 ± 11, respectively. Peak HR for groups R and F was 171 ± 4 and 155 ± 5, which were lower than the HR for group C (p < 0.001). Although NE concentrations at rest, during warm-up, and at VT were significantly greater in groups R and F (p < 0.05), there were no significant differences in the NE concentrations at peak exercise. Peak HR correlated with %pVO₂ (p < 0.001). The ratio of the increase in HR to NE from rest to VT was significantly lower in groups R and F than in group C (p < 0.001) and correlated with %pVO₂ (r= 0.80; p < 0.001). These data suggest that sympathetic nervous activity in groups R and F is increased at rest and during mild to moderate exercises, and reduced sinus node sensitivity to NE may be partly responsible for the abnormal HR response during exercise of patients with uncorrected congenital heart disease.     

Development of Superior Vena Cava to Pulmonary Vein Fistulae Following Modified Fontan Operation: Case Report of a Rare Anomaly and Embolization Therapy

A patient with double inlet single ventricle, L-transposition of the great arteries, and atrioventricular valve regurgitation developed progressive heart failure necessitating modified Fontan operation at 16 months of age. The procedure included replacement of the atrioventricular valve and pacemaker insertion. Eight years following the operation, the patient developed progressive cyanosis. Catheterization confirmed the presence of fistulae from the superior vena cava and innominate vein to the pulmonary veins as the cause of cyanosis. Coil embolization of several fistulae was performed successfully at catheterization. Contributing factors for fistula formation in Fontan patients are discussed, and therapy is reviewed.     

Fenestration of Extracardiac Fontan and Reversal of Protein-Losing Enteropathy: Case Report

We describe a patient with protein-losing enteropathy who presented 6 months after undergoing a modified Fontan operation. After failing to respond to medical therapy, the Fontan tunnel was fenestrated by catheter intervention with immediate improvement and resolution of hypoproteinemia and enteric protein loss.     

Pulmonary thrombo-embolism in nephrotic syndrome treated with tissue plasminogen activator

We describe the case of a boy with steroid sensitive nephrotic syndrome and left pulmonary artery thrombo-embolism. Clinical presentation initially suggested sepsis and respiratory signs were minor. Treatment with tissue plasminogen activator infused into the pulmonary artery was successful. Conclusion Pulmonary thrombo-embolism should be considered in unwell children with nephrotic syndrome. Received: 6 September 1996 / Accepted: 17 December 1996     

Use of Tissue Plasminogen Activator for Femoral Artery Thrombosis Following Transcatheter Coil Occlusion of Patent Ductus Arteriosus

Femoral artery thrombosis is an uncommon but potentially serious complication following pediatric cardiac catheterization. Management options include heparin infusion, thrombolytic therapy, and surgical thrombectomy. The use of thrombolytic agents following coil occlusion of shunts, collaterals, and patent ductus arteriosus (PDA) may be successful in resolving the femoral arterial thrombosis but may also reopen the device-occluded vessel. We report the successful use of tissue plasminogen activator for management of femoral artery thrombosis in a child following transcatheter PDA coil occlusion in which the PDA remained occluded.     

Left-Side Pulmonary Vein Obstruction After Arterial Switch Operation in Infants with D-Transposition of the Great Arteries

We describe two cases of left-side pulmonary vein obstruction observed after the arterial switch operation (Jatene) for D-transposition of the great arteries. This appears to be related to left-sided pulmonary vein obstruction occurring coincidently with D-transposition of the great arteries, rather than a consequence of arterial switch operation.     

Redirection of the Hepatic Venous Flow for the Treatment of Pulmonary Arteriovenous Malformations after Fontan Operation

A 45-month-old boy who had complex cardiac anomalies with interrupted inferior vena cava and polysplenia underwent extracardiac Fontan operation. He redeveloped deep cyanosis postoperatively. Cardiac catheterization showed a preferential flow of the hepatic venous blood to the right lung and arteriovenous malformations (PAVMs) in the left lung. He underwent revision of the conduit to attain balanced hepatic venous drainage with subsequent regression of the PAVMs and disappearance of cyanosis. Our experience shows that deficiency of hepatic venous flow played a crucial role in the development of PAVMs and that redistribution of the flow retained potential to ameliorate the pathological condition.     

Adverse Hemodynamic Effects Observed with Inhaled Nitric Oxide After Surgical Repair of Total Anomalous Pulmonary Venous Return

The following is a case report of a 1-month-old patient who developed adverse hemodynamic sequelae during the use of nitric oxide (NO) in the postoperative period for pulmonary hypertension after correction of total anomalous pulmonary venous return. At the time of diagnosis, the patient had evidence of systemic right ventricular pressures estimated by continuous-wave Doppler. He was sedated and paralyzed for hyperventilation in preparation for surgery and underwent pulmonary vein confluence to left atrial anastomosis. Postoperative pulmonary hypertension was managed by hyperventilation, sedation, and paralysis until a sudden onset of systemic-level pulmonary pressure required NO therapy. Satisfactory results were obtained in minutes, but a rebound pulmonary hypertension occurred with concomitant systemic hypertension and no radiographic changes. We suspected left atrial hypertension secondary to a sudden increase in pulmonary blood flow to an noncompliant left ventricle. Discontinuation of NO resulted in stabilization of the hemodynamic profile of the patient and he continued to be managed with paralysis, hyperventilation, and sedation. Based on this experience we suggest that NO should be used with caution in patients with obstructive lesions at the atrial level prior to surgery (mitral valve stenosis and cor triatriatum) or in patients with a poorly compliant left ventricle (cardiomyopathy and left ventricular dysfunction). These entities are unable to tolerate a sudden increase in pulmonary blood return thus creating paradoxical pulmonary hypertension.     

Exercise Performance Following Repair of Hypoplastic Left Heart Syndrome: A Comparison with Other Types of Fontan Patients

Reports of exercise performance after Fontan surgery for hypoplastic left heart syndrome (HLHS) are lacking. We compared the exercise performance of total cavopulmonary connection type (TCPC) of Fontan subjects with HLHS (group 1, n= 7) to those not requiring a Norwood procedure having a systemic right ventricle (group 2, n= 6) or a systemic left ventricle (group 3, n= 8). The subjects underwent assessment of resting pulmonary mechanics followed by maximal exercise testing with a bicycle or treadmill protocol. ECG, oxygen consumption, and carbon dioxide production were measured continuously. There was not a significant difference seen between HLHS and the comparison groups for the following parameters: maximum heart rate, maximum oxygen consumption, respiratory exchange ratio, breathing reserve, and arterial oxygen saturation at rest or exercise. Exercise performance in the TCPC type of Fontan patients was comparable regardless of ventricular morphology or surgical approach.     

Course of Right and Left Ventricular Function in Patients with Pulmonary Insufficiency After Repair of Tetralogy of Fallot

Surgical repair of tetralogy of Fallot (TOF) frequently results in pulmonary valve insufficiency. Nevertheless, no serial information is available on the long-term impact of the valvular insufficiency on right and left ventricular function. Right and left ventricular ejection fraction was measured serially by radionuclide angiocardiography in 21 patients with at least moderate pulmonary insufficiency after repair of TOF. A baseline study was obtained an average of 1.2 years after repair, and a follow-up study was performed an average of 10.2 years after surgery. Changes in ventricular function over time and deviations from the normal range were analyzed. At baseline evaluation the mean right ventricular ejection fraction (RVEF; 0.52 ± 0.10) and left ventricular ejection fraction (LVEF; 0.68 ± 0.10) were normal. At the time of follow-up the mean RVEF had significantly decreased to 0.45 ± 0.09 (p < 0.01). The mean LVEF had decreased to 0.60 ± 0.11 (p < 0.02). This change was independent of the RVEF (r=−0.13). Eleven patients (52%) had an abnormal RVEF or LVEF at follow-up. Nineteen patients (90%) showed a decrease of 0.05 or more in RVEF, LVEF, or both between studies. These data suggest a negative impact of long-standing pulmonary insufficiency on right and left ventricular systolic function after repair of TOF. Therefore, continued surveillance of biventricular function in this patient population appears warranted.     

Pulmonary Cavernous Hemangiomatosis Treated with Interferon Alfa-2a

Pulmonary hemangiomatosis is a rare, usually fatal disorder characterized by diffuse proliferation of blood vessels within the thorax. We describe a 7-year-old boy with cavernous-type pulmonary hemangiomatosis successfully treated with interferon alfa-2a. He presented with respiratory distress and hemoptysis that were alleviated during a 2-year follow-up period.     

Fatal Rupture of an Acquired Aneurysm of the Pulmonary Artery: Rare Complication After Surgical Palliation of Tricuspid Atresia

We report the case of a young woman who died from rupture of an aneurysmal dilatation of the left pulmonary artery. She suffered from tricuspid atresia type Ib and underwent a classic Glenn anastomosis at the age of 11 months; at 11 years a direct laterolateral anastomosis was constructed between the ascending aorta and the left pulmonary artery rather than a Fontan procedure for technical reasons. She subsequently developed severe pulmonary hypertension and an aneurysmal dilatation of the left pulmonary artery and was refused any further surgical correction.     

Exercise Testing in Children with Pulmonary Valvar Stenosis

Pulmonary valvar stenosis with intact ventricular septum is a common anomaly. This lesion poses a fixed obstruction to the right ventricular outflow. The right ventricle ejects the entire cardiac output across the stenotic valve. Right ventricular systolic pressure and oxygen demand are increased at rest and more so with exercise. Exercise tolerance in children and adults with mild valvar pulmonary stenosis is nearly normal, but is diminished in those with moderate and severe stenosis, indicating impaired ability to sustain adequate cardiac output. Following relief of stenosis, cardiac performance improves in children, but remains abnormal in adults. This appears to be related to postoperative resolution of right ventricular hypertrophy in children, whereas myocardial fibrosis may explain the lack of improvement in adults.