Derek A. Fyfe

Anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome) in adult patients: Long-term follow-up after surgery

Congenital anomalous origin of the left coronary artery arising from the pulmonary artery, or the Bland-White-Garland syndrome (BWGS), is an uncommon but frequently lethal lesion of both children and adults.’ Although approximately 80 % to 90% of patients develop congestive heart failure and die in infancy, some patients may present as adolescents or adults.*z3 Although often asymptomatic, many adults with BWGS demonstrate evidence of previous myocardial infarction and frequently exhibit ischemic patterns when evaluated with stress electrocardiography or stress thallium-201 myocardial imaging.4-6 Several reports of recurrent arrhythmias7 or sudden death2s8 in such patients have been published. Most clinicians recognize the need for surgical intervention. Surgical approaches have included reimplantation of the anomalous left coronary artery from the pulmonary artery to the aorta,g ligation of the anomalous left coronary artery from inside the pulmonary artery,‘O or ligation of the left coronary artery combined with saphenous vein grafting to the anterior descending coronary artery.” No data are currently available which demonstrate the superiority of one surgical approach over another in adult patients. This report describes the presentation, evaluation, and long-term follow-up in 10 adult patients with BWGS following various methods of surgical repair.

Transesophageal echocardiography detects thrombus formation not identified by transthoracic echocardiography after the fontan operation

Transesophageal echocardiography demonstrated six instances of venous thrombus formation in the inferior vena cava, right atrium and caval-pulmonary anastomosis region in four children after a modified Fontan operation. Transthoracic surface echocardiography failed to identify these thrombi in five of the six cases because of the posterior location of the thrombus or imaging interference from surgical hardware. These thrombotic episodes occurred 2 days to 5 years after the Fontan operation in children 25 to 168 months of age. Clinical features of compromised cardiac performance with cyanosis or inadequate perfusion were present during four of the six episodes. In two patients, thrombi occurred around transvenous permanent atrial pacing leads. Therapy to eliminate thrombus included surgery (two cases), anticoagulation with warfarin (three cases) and streptokinase thrombolysis (one case). Disappearance of the thrombus was confirmed by transesophageal study in three of the four cases with follow-up echocardiography. Transesophageal echocardiographic demonstration of atrial and pulmonary thrombi that could not be seen by transthoracic imaging suggests that these thrombi occur with greater frequency in patients who have undergone the Fontan operation than was previously suspected.

Right ventricular outflow reconstruction with cryopreserved homografts in pediatric patients: Intermediate-term follow-up with serial echocardiographic assessment

OBJECTIVESnThis study was performed to assess by echocardiography the intermediate-term outcome of cryopreserved homografts employed in pulmonary outflow reconstruction in children and to validate the reliability of Doppler echocardiography in their evaluation.nnnBACKGROUNDnCryopreserved homografts have become the most widely used pulmonary conduits. Previous reports have shown the occurrence of homograft regurgitation in the immediate postoperative period and the propensity of regurgitation to progress. Although Doppler echocardiography has been useful in assessing extracardiac valved conduit stenosis, its reliability in assessing a large series of cryopreserved homografts has not been documented.nnnMETHODSnEchocardiograms of 41 patients (43 homografts) who underwent operations between December 1986 and October 1992 were retrospectively reviewed. The median age of patients at operation was 37.5 months (range 3 to 333), and the median duration of follow-up was 28.5 months (range 1 to 68). Homograft regurgitation was classified on a scale of 0 to 4+. Pressure gradients across the homografts measured in 23 catheterizations were correlated with corresponding echocardiographic gradients.nnnRESULTSnRegurgitation: Homograft regurgitation occurred in 100% of patients at follow-up. Progression of severity > 2 grades occurred during follow-up in 35% and was associated with operation before age 18 months (p < 0.002) and stenosis progression (p < 0.05) but not with homograft type (aortic or pulmonary). These data predict that 50% of patients operated on before 18 months of age will have severe regurgitation by 15 months postoperatively compared with only 15% operated on after 18 months. Stenosis: At follow-up, 51% of homografts had a stenotic gradient > or = 25 mm Hg predominantly at the distal anastomosis, and stenosis progression was related to young age at operation (< 18 months, p < 0.005) and small conduit size (p < 0.01). Fifty percent of conduits implanted before age 18 months could be predicted to stenose by 21.8 months compared with only 5% of those implanted after age 18 months. The gradient measured from Doppler echocardiography correlated well with the catheterization gradient (r = 0.86).nnnCONCLUSIONSnCryopreserved homograft dysfunction is frequent and progressive. Young age at operation (< 18 months) predicts more rapid deterioration. Doppler echocardiography is reliable in assessing the systolic gradients across homografts. Serial echocardiographic assessment in the follow-up of these patients accurately characterizes these problems.

Hemi-front operation in surgery for single ventricle: A preliminary report☆

Mortality after Fontan operation is related to risk factors like ventricular hypertrophy, pulmonary artery deformity, and young age (infancy). Preliminary procedures may improve Fontan results. The hemi-Fontan operation includes atriopulmonary anastomosis and correction of all anatomical risk factors, but an atriopulmonary patch directs superior vena caval flow into both pulmonary arteries and inferior vena caval flow into the ventricle, thus maintaining cardiac output (modified Glenn physiology). We performed 17 hemi-Fontan procedures in 16 patients, 14 primarily (median age, 9 months) and 3 for takedown of a Fontan operation. The 14 primary operations were for hypoplastic left heart syndrome (5), pulmonary atresia with intact ventricular septum (4), and other (5). All patients had multiple risk factors. Extubation was at 18 hours (median), chest tube removal was at 3 days, and hospital discharge was at 8 days postoperatively. Important complications included subglottic stenosis, transient diaphragmatic paralysis, pulmonary artery stenosis and thrombosis requiring reoperation, and transient ventricular fibrillation. One patient required hemi-Fontan takedown, and this patient later (3 months postoperatively) became the only death. Fontan take-downs have had a high mortality rate. In 3 patients who tolerated Fontan operation poorly, converting Fontan to hemi-Fontan abruptly reversed the downhill course. For these patients, the operation was life-saving. Hemi-Fontan operation is safe and well-tolerated, even in infants, provides the advantages of modified Glenn physiology before Fontan operation, and may be especially useful for Fontan takedown after failed Fontan.

The utility of transesophageal echocardiography during and after Fontan operations in small children

Transesophageal echocardiography with Doppler examination was performed intraoperatively in 19 children undergoing modified Fontan operations and in 10 patients postoperatively. Comparisons were made with results of intraoperative epicardial imaging (9 patients) and with postoperative transthoracic imaging (10 patients). Transesophageal echocardiography optimally visualized atriopulmonary and cavopulmonary anastomoses. Epicardial echocardiography was successful in only three of nine patients. Intraoperative transesophageal echocardiography showed residua in 8 of 19 studies and led directly to surgical revision or medical therapy. These residua included stenosis of the cavopulmonary anastomosis (1 patient), unsatisfactory atrial fenestration (2 patients), patent ductus arteriosus (1 patient), residual cavoatrial shunting (1 patient), atrial thrombi (1 patient), and poor ventricular function (2 patients). Results of examination in the postoperative intensive care unit showed significant abnormalities in 4 of 10 patients. This study demonstrates that transesophageal echocardiography provides unique anatomic and physiologic information during and after modified Fontan operations in small children and therefore may have significant impact on patient management.

New approaches to treatment of incessant ventricular tachycardia in the very young.

It was assumed that the availability of new antiarrhythmic drugs and new surgical techniques might allow medical or nonexcisional surgical treatment in many young children with incessant ventricular tachycardia. Fourteen infants and young children less than 5 years of age were evaluated and treated for incessant ventricular tachycardia. Medical treatment was pursued up to the use of amiodarone with a type Ib or Ic antiarrhythmic drug unless the patient became hemodynamically unstable. Patients underwent surgery when these drug regimens failed or when moderate congestive heart failure was present. Seven patients were successfully treated medically and seven underwent surgical treatment. Of those treated surgically, five had cryothermic lesions and two had excisions. Five of the surgically treated patients required temporary additional medical treatment. Follow-up ranged from 12 to 53 months (mean 28). Eleven of the 14 patients are currently not taking any antiarrhythmic medication. No patient required a pacemaker, none received anticongestive medications and none died.

Clinical spectrum of venous thrombi in the Fontan patient

Abstract Venous thrombus formation is a known complication after the Fontan procedure. To characterize the clinical presentation, diagnostic methods, treatment regimens and outcome of this disease process, we retrospectively reviewed the records of 7 patients who developed systemic venous or pulmonary arterial thrombosis after modifications of the Fontan operation. Clinical features were analyzed to identify “risk factors” that may predispose to thrombus formation.

Double-chambered right ventricle

Thirty (10.8%) of 279 patients undergoing correction of a ventricular septal defect (VSD) from January, 1972, to September, 1986, also had a double-chambered right ventricle (DCRV). Age at operation ranged from 1.3 to 18.8 years (mean, 6.7 +/- 4.5 years [+/- standard deviation]). Seventeen patients were male, and 13 were female. Two-dimensional echocardiography was used after 1978 in the initial evaluation of 20 patients; however, the diagnosis of DCRV was made with the use of subcostal views only since 1984 in 4 of 5 patients. Surgical correction consisted of closure of the VSD and resection of anomalous muscle bundles through a right ventriculotomy (28 patients), and right atriotomy (2 patients). All patients survived and are asymptomatic 4.2 +/- 3.4 years following operation. Six patients have undergone catheterization postoperatively and 8 patients had intraoperative pressure recordings. The mean preoperative ratio of right ventricular to left ventricular pressures was 0.67 +/- 0.22 compared with 0.34 +/- 0.15 postoperatively (p less than .001). In 2 patients, DCRV was not recognized preoperatively or at VSD closure through a right atriotomy, and reoperation was necessary after DCRV was demonstrated at postoperative catheterization. DCRV may occur in approximately 10% of patients undergoing correction of VSD. Careful evaluation of echocardiographic and catheterization data preoperatively and careful evaluation of the anatomy intraoperatively are necessary so that DCRV not be overlooked, especially because most VSDs are now closed through the right atrium. Successful correction of VSD and DCRV is associated with excellent long-term results.

Doppler echocardiographic evaluation of streptokinase lysis of thrombosed right-sided St. Jude Medical valves in patients with congenital heart defects

Four episodes of St. Jude Medical prosthesis leaflet thrombosis were serially evaluated by two-dimensional and Doppler echocardiography during treatment with streptokinase. Three patients aged 4, 11, and 24 years with congenital heart disease had St. Jude Medical valves in pulmonary positions (two cases) for tetralogy of Fallot and in the tricuspid position (one case). The duration of thrombosis was not known in any patient. Leaflet immobility and its resolution were demonstrated by echocardiography and were confirmed fluoroscopically. Continuous wave Doppler echocardiography showed abnormal stenotic gradients in thrombosed valves that were reduced after thrombolysis. These studies demonstrate the utility of two-dimensional and Doppler echocardiography in serial evaluation of prosthetic pulmonary and tricuspid valve thrombosis during thrombolysis.

Transesophageal Echocardiography for Congenital Heart Disease

Surface echocardiographic imaging of small children is routinely successful in defining anatomical details and Doppler flow patterns with even the most complex congenital cardiac malformations. However, in larger children or adults, imaging is frequently limited. A recent expansion of the role of echocardiography is intraoperative epicardial imaging. Epicardial and postoperative imaging, however, have significant limitations. To avoid some of these limitations, transesophageal echocardiography has increasingly been used in the arena of congenital heart disease. The more recent development of small sized gastroscopic probes has allowed transesophageal echocardiographic assessment of congenital heart disease in children down to newborn size. As detailed studies of individual lesions are reported, it has become clear that the mere presence of a congenital heart defect is not an indication for transesophageal echocardiography in most children if imaging can be accomplished by surface examination. However, transesophageal echocardiography may be indicated for the intraoperative or postoperative assessment of that defect, particularly when repair has been difficult or is known to be associated with significant residual abnormalities. Cardiac structures encountered with horizontal and vertical imaging plane transducers have been described and should be completely familiar to the examining echocardiographer. (ECHOCARDIOGRAPHY, Volume 8, September 1991)