Lactate dehydrogenase isozymes in human cardiac transplantation.

Lactate dehydrogenase (LDH) isozyme activities have been followed in 17 human cardiac allografts. A pattern of abnormality associated with cardiac rejection during the first month after operation has been determined: (i) LDH-1 activity is greater than LDH-2 activity; (ii) LDH-1 activity is greater than 35 percent of total LDH activity; and (iii) LDH-1 activity is greater than 100 international units. The LDH-1 abnormality helps to meet the need for an index of cardiac rejection during the early weeks after operation when the electro-cardiogram is least reliable.

Severe Takayasu's arteritis of the pulmonary arteries: report of a case with successful surgical treatment.

Although involvement of the aorta and its large branches is more common, Takayasu's arteritis involving the pulmonary arteries is well recognized. This report describes an adolescent girl with an uncommonly severe form of Takayasu's arteritis involving the pulmonary arteries. A successful surgical treatment is presented.

Two-dimensional echocardiographic assessment of complications involving the Ionescu-Shiley pericardial valve in the mitral position.

The function of the Ionescu-Shiley bovine pericardial xenograft in the mitral position was investigated in 70 patients by two-dimensional echocardiography. Echocardiographic data from 21 patients with suspected bioprosthetic dysfunction and 49 patients with normal clinical findings were analyzed in a double-blind fashion. Confirmation of cardiovascular status was obtained by means of cardiac catheterization, surgery, autopsy or other techniques in 19 of the 21 symptomatic patients. Two-dimensional echocardiography correctly evaluated bioprosthetic function in 98% of the patients. Six of the seven patients with proven xenograft dysfunction demonstrated echocardiographic evidence of malfunction. There was one false negative evaluation but no false positive results. Valve dysfunction included endocarditic mass lesions, mitral regurgitation resulting from dehiscence of a leaflet or the sewing ring or mitral stenosis due to calcification or malposition of the valve. This experience indicates that two-dimensional echocardiography is useful for assessing the Ionescu-Shiley mitral bioprosthesis, particularly in those patients who develop nonspecific symptoms.

Coronary revascularization in the elderly patient.

A total of 1,275 elderly patients (70 years and older) underwent coronary artery bypass alone from 1970 to 1981. The percent of elderly patients who underwent coronary bypass surgery alone increased from 2.04% in 1971 to 8.2% in 1981. Most of the patients had severe, disabling or unstable angina pectoris. The overall early mortality rate was 5.8%. The early mortality rate was 13.9% in the first group (1970 to 1975) of 158 patients compared with 4.7% in the second group (1976 to 1981) of 1,117 patients. An average of 3.1 bypass grafts per patient were implanted. On follow-up examination, angina was relieved or decreased in 89% of the patients. The 5 year survival rate was 80.6% and the 10 year survival rate was 44.1%, with an average attrition of 3.9 and 5.6%/year, respectively. It is concluded that elderly patients are high risk surgical candidates, yet the risk has decreased progressively because of improved techniques of medical and surgical management and myocardial preservation. This decreasing operative mortality rate provides evidence that when medical management of the elderly patient with severe angina fails, coronary artery bypass becomes a successful alternative.

Surgical management of refractory supraventricular tachycardia in infants and children.

Sixty-seven children underwent attempted surgical correction of refractory supraventricular arrhythmias using a combination of intraoperative electrophysiologic mapping followed by surgical division or cryoablation of an aberrant conduction pathway or atrial ectopic focus. The patients ranged in age from 4 months to 18 years (mean 11.4 years). Fifty-five patients (82%) had an abnormal conduction pathway crossing the atrioventricular junction (Kent bundle). Thirty-six (65%) of these 55 patients had classic Wolff-Parkinson-White syndrome with a delta wave of pre-excitation on the surface electrocardiogram. Nineteen (35%), however, demonstrated only retrograde conduction across the Kent bundle and had a normal surface electrocardiogram when tachycardia was not present. Kent bundles were isolated to the following locations: right anterior or lateral in 19 (34.5%), left posterior or lateral in 22 (40%), posteroseptal in 10 (18%), anteroseptal in 2 (4%) and both right and left in 2 (4%). Follow-up evaluation of as long as 8 years (mean 34.9 months) has shown seven immediate failures and one late recurrence of arrhythmia (14.5%). Recent refinements in technique and the use of cryoablation for septal aberrant pathways have improved these results. There have been two failures (8%) in the last 25 attempts. Twelve patients underwent surgery for an atrial ectopic focus by the following techniques: cryoablation in seven patients, excision in one patient and both excision and cryoablation in four patients. At a mean follow-up of 16.6 months, there was one late recurrence in the group with an atrial ectopic focus.(ABSTRACT TRUNCATED AT 250 WORDS)

Computer-guided surgery for tachyarrhythmias in children: current results and expectations.

OBJECTIVES: The purpose of this report is to summarize our entire surgical experience in the treatment of tachyarrhythmias in children. We emphasize our application of a newer computerized mapping system for use in both the electrophysiology laboratory and the operating room to localize points of activation of the tachyarrhythmias. BACKGROUND: A retrospective review was undertaken to examine the results of operative procedures in 290 children undergoing surgical treatment for tachyarrhythmias from 1977 to the present. METHODS: Operative procedures were performed in 290 children and consisted of the following: surgical ablation of accessory pathways of the Kent bundle type (210 children); surgery with cryoablation for atrial ectopic tachycardia (35 children); surgical excision or cryoablation, or both, for ventricular tachycardia (26 children); cryoablation for the permanent form of junctional reciprocating tachycardia (15 children) and atrioventricular (AV) node reentrant tachycardia (4 children). RESULTS: The surgical cure rate for accessory pathway tachycardia in the era before computerized mapping was 80% (41 patients) in the period from 1977 to 1982 and 95% (86 patients) in the period from 1982 to 1988. This rate improved to 100% (83 patients) after the advent of the computerized mapping technique. These improved results are probably due to a combination of factors, including increasing experience in electrophysiologic mapping and surgery, and cannot be attributed to the computerized mapping system alone. Surgical cure or major improvement in symptoms was documented in 33 (94%) of 35 patients with atrial ectopic tachycardia. Surgical cure was accomplished in 25 (96%) of 26 patients with the complex form of ventricular tachycardia. In 19 patients with the permanent form of junctional reciprocating tachycardia and the more typical AV node reentrant tachycardia, the surgical cure rate was 100%. CONCLUSIONS: In all forms of supraventricular reentrant tachycardia that occur in children, preoperative computerized mapping techniques combined with intraoperative computerized mapping and surgical ablation can eliminate tachycardia at a success rate of close to 100%. Computerized mapping techniques are less accurate in patients with atrial ectopic tachycardia because of multiple foci and a broader surface area to be mapped. This experience demonstrates that excellent results can be achieved in the surgical treatment of tachyarrhythmias in children.

Incessant ventricular tachycardia in infants: myocardial hamartomas and surgical cure.

Infants with incessant ventricular tachycardia (occurring greater than 10% of the day) have generally been described in pathologic studies. This report describes 21 patients with incessant ventricular tachycardia present greater than 90% of the day and night; the age at diagnosis ranged from birth to 30 months (mean 10.5 months). The most common clinical presentation was cardiac arrest (11 patients, in 5 after digitalis for presumed supraventricular tachycardia); another 6 patients had congestive heart failure and 4 were asymptomatic. Three patients had coexisting Wolff-Parkinson-White syndrome. The rate of incessant ventricular tachycardia ranged from 167 to 440 (mean 260 beats/min) and the QRS duration from 0.06 to 0.11 second. The most common electrocardiographic (ECG) pattern (10 of 21) was right bundle branch block with left axis deviation, but other right and left bundle branch block patterns were observed. Conventional and investigational antiarrhythmic agents (nine patients received amiodarone) failed to eliminate incessant ventricular tachycardia in all. Electrophysiologic studies localized incessant ventricular tachycardia to the left ventricle in 17 (to the apex in 2, the free wall in 9 and the septum in 6) and to the right ventricular septum in 4. No structural abnormalities were found on the echocardiogram or angiocardiogram. All 21 patients had surgery at an age of 3.5 to 31 months (mean 16). In 15 a tumor was found: 13 myocardial hamartomas (9 discrete, 4 diffuse throughout both ventricles) and 2 rhabdomyomas (1 multiple). Myocarditis was found in one patient (the oldest). In four, only myocardial fibrosis was found; results of one biopsy were normal.(ABSTRACT TRUNCATED AT 250 WORDS)

Hemodynamic compromise (tricuspid stenosis and insufficiency) caused by an unruptured aneurysm of the sinus of Valsalva.

A patient is described with an unruptured aneurysm of the noncoronary sinus of Valsalva that occupied the right ventricular inflow tract and caused dynamic tricuspid stenosis and insufficiency. Results of two-dimensional echocardiography delineated the anatomy of the aneurysm and pulsed Doppler examination provided evidence that the aneurysm was unruptured. The unruptured aneurysm was resected successfully.

Pacemaker treatment of sick sinus syndrome in children.

The sick sinus syndrome is being recognized with increasing frequency in children. Although it is sometimes benign, it can be serious or have fatal consequences. Fifty-one patients (mean age 10.5 years) underwent permanent cardiac pacing for sick sinus syndrome. Twenty patients had epicardial ventricular pacing and 12 had an epicardial atrial implant. Seven had endocardial atrial pacing, six epicardial atrioventricular (AV) sequential pacing, four epicardial universal pacing and two endocardial universal pacing. Of the 49 symptomatic patients, 45 had relief of symptoms. Eleven of 18 patients with associated tachyarrhythmias had amelioration of their tachycardia. There were no early but two late deaths unrelated to the pacemakers. Seven patients during a mean follow-up period of 26 months required reoperation for pacing lead or sensing problems. Permanent pacing for sick sinus syndrome in children is a safe and symptomatically effective procedure.

Late deterioration in tetralogy of Fallot: unusual findings and successful correction.

A patient with tetralogy of Fallot who underwent a successful Blalock shunt procedure as a child was evaluated 28 years later because of clinical deterioration. Results of cardiac catheterization and angiography disclosed, in addition to a functioning shunt, evidence of an infundibular, subvalvular tumor and a large anastomosis between the left circumflex coronary artery and the bronchial arteries of the right lung with the possibility of "coronary steal". At operation, the Blalock and coronary-to-bronchial artery anastomoses were ligated; and total correction was performed including resection of the pulmonary valve and infundibulum, excision of the tumor, closure of the ventricular septal defect, and application of an outflow patch to enlarge the pulmonary annulus. The patient was discharged nine days after surgical correction.

Exacerbation of cyclosporine toxicity by concomitant administration of erythromycin.

Cyclosporine (CsA), an immunosuppressive drug widely used in clinical organ transplantation, causes a variety of side effects, including parenchymal complications of nephrotoxicity and hepatotoxicity. Erythromycin ethinylsuccinate (EES), a macrolide antibiotic frequently administered to transplant patients afflicted with pneumonias caused by Mycoplasma pneumoniae and Legionella pneumophila, markedly potentiated parenchymal drug toxicity in nine (three renal and six cardiac) CsA-treated allograft recipients. The mean and median blood urea nitrogen (BUN), creatinine, and total bilirubin increased upon initiation of EES treatment: in the renal recipients from 27, 1.7, and 0.5 mg/dl, respectively, before, to a mean and median of 81/101, 8.3/3.9, and 2.1/1.2 mg/dl during, and to 72/22, 1.9/1.7, and 0.6/0.5 mg/dl after cessation of EES treatment. The median serum radioimmunoassay (RIA)-determined CsA trough value of 147 ng/ml prior, rose to a zenith of 1125 ng/ml during, EES therapy. In the six cardiac recipients, the mean and median BUN, creatinine, and total bilirubin of 51/45, 1.5/1.3, 1.2/1.3 mg/dl, respectively, before, rose to 100/91, 3.7/3.6, and 2.3/2.1 mg/dl during, and fell to 49/44, 1.8/2.1, and 1.0/0.8 mg/dl after, cessation of EES. The mean serum CsA trough value of 185 ng/ml rose to 815 ng/ml during EES administration. Since EES and CsA are both metabolized by the hepatic cytochrome P450 mixed-function oxidase system, simultaneous use of these two drugs may decrease CsA metabolism, with consequent elevation of blood levels and induction of CsA toxicity. Therefore, blood level monitoring and careful regulation of CsA dose are necessary, in order to achieve the safe use of EES in transplant recipients.

Postoperative pericardial effusion and its relation to postpericardiotomy syndrome.

Postpericardiotomy syndrome often delays recovery from cardiac operation. Pericardial effusion is an important sequela of postpericardiotomy syndrome. To define the relationship between postpericardiotomy syndrome and pericardial effusion, we performed four to seven serial echocardiograms (echoes) between postoperative day 0 and postoperative day 10 in 40 children who had operation requiring pericardiotomy. We also performed daily physical examination on each patient for clinical evidence of postpericardiotomy syndrome. Echocardiographic signs of pericardial effusion developed in 21/40 patients (53%). The effusion was present by postoperative day 5 in 19/21 patients. Clinical signs of postpericardiotomy syndrome occurred in 18/40 patients (45%). Among the 18 patients with postpericardiotomy syndrome, 16 had echocardiographic evidence of pericardial effusion. Thus the sensitivity of the echocardiogram was 89%. The pericardial effusion always developed prior to and persisted through clinical symptoms. We conclude that (1) pericardial effusion is present in over 50% of the patients following cardiac surgery, (2) it develops early in the postoperative period, and (3) once present, the effusion is persistent and frequently followed by clinical evidence of postpericardiotomy syndrome.

Thoracoabdominal ectopia cordis with mosaic Turner's syndrome: Report of a case.

A child was treated for thoracoabdominal ectopia cordis and an associated chromosomal defect. Contrary to most cases in which death is due to the externally situated heart and abdominal viscera, this patient died from congenital heart disease.

Aortic implantation of anomalous left coronary artery arising from pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery is a rare and usually fatal congenital malformation. Most surgical attempts to correct this anomaly have been unsuccessful, particularly those in infants. Recently, four patients underwent reimplantation of an anomalous left coronary artery into the aorta; all four survived the operation and are asymptomatic. This technique of reimplantation of the aberrant vessel into the aorta appears to be prefereble to other methods of repair, especially since it can be applied in any patient with the anomaly regardless of age or size. Because of the poor prognosis for patients with anomalous left coronary artery, surgical intervention should be made as soon as the diagnosis is made.

Association of ostium secundum atrial septal defects with mitral valve prolapse.

Severe prolapse of the mitral valve leaflets was seen at left ventricular angiography in 16 of 92 patients with a secundum type atrial septal defect studied prospectively from 1970 to 1974. The patients were aged 15 to 69 years; angioplasty or mitral valve replacement was carried out in nine. In 9 of 122 patients aged 15 to 55 years who were operated on for closure of a secundum type atrial septal defect between 1956 and 1969, mitral regurgitation due to prolapse but with intact chordae tendineae was seen at operation. In three of these patients chordal rupture was seen at a second operation 2 to 6 years later. The outlook in the syndrome of mitral valve prolapse may be less benign than is usually believed.

Surgical treatment of supraventricular tachycardia in infants and children.

The technique, indications and results of surgical division of accessory atrioventricular connections in 10 infants and children with drug-resistant supraventricular tachycardia are described. The patients ranged in age from 6 months to 15 years. Four patients had associated congenital heart disease. Division of accessory connections were performed on free wall pathways in nine patients (seven right atrial, two left atrial) and on a septal pathway in one patient. Four patients had both anterograde and retrograde conduction over the accessory connection (manifest Wolff-Parkinson-White conduction) whereas six had only retrograde conduction (concealed Wolff-Parkinson-White conduction). The manifst Wolff-Parkinson-White conduction was abolished by surgical division in all four patients. In 8 of the 10 patients the procedure stopped the attacks of paroxysmal supraventricular tachycardia for follow-up periods ranging from 9 months to 3 1/2 years; no patient receives medication to date.

Hemodynamic observations in patients with left ventricle to aorta conduit.

Fourteen patients who had a left ventricular apex to abdominal aorta composite conduit implanted for relief of severe left ventricular outflow tract obstruction were studied at cardiac catheterization 7 days to 19 months postoperatively (median 12 days). Analysis of pressure, angiographic and blood flow velocity data showed the following: (1) The left ventricle to aorta pressure gradients were reduced from an average preoperative value of 100.6 mmHg (range 54 to 140) to an average of 22.1 mm Hg (range 0 to 60) postoperatively; (2) postoperative left ventricular end-diastolic volume averaged 87.5 percent of normal (range 51 to 146); (3) cardiac index and ejection fraction were normal postoperatively in all but one patient. Left ventricular and aortic angiography revealed blood flow through both the ascending aorta and conduit in all patients and retrograde flow of blood from the conduit to the aortic arch in four. An average of 36 percent (range 16 to 50) of the left ventricular output was ejected through the conduit. It appears that this operation is effective in relieving severe and otherwise inoperable left ventricular outflow tract obstruction and in preserving or improving left ventricular function and aortic hemodynamics.

Management of surgical complete atrioventricular block in children.

Because there is disagreement concerning the efficacy of and indication for permanent pacemaker implantation in children with postoperative complete (third degree) atrioventricular (A-V) block, experience in the management of this problem at one institution was reviewed. Thirty-four patients with postoperative complete atrioventricular block were identified. They ranged in age from 4 months to 22 years and in weight from 4 to 60 kg and were evaluated from 1 month to 20 years postoperatively. Complete A-V block developed within 24 hours of operation in 28 of the 34 patients. A permanent pacemaker was implanted in 13 of the 28. Death occurred in 4 of these 13 patients and in 5 of the remaining 15 patients who did not have an artificial permanent pacemaker. Complete A-V block developed later than 1 day (2 days to 4 months) postoperatively in 6 of the 34 patients; all 6 of these patients survived, and only 3 required permanent pacemaker implantation. Intracardiac electrophysiologic studies were performed by 14 of the 34 patients. The site of complete block was above the His bundle in 5, within the His bundle in 2, and below the His bundle in 4; it was undetermined in 3. The results of intracardiac electrophysiologic studies are important in delineating the natural history of surgically induced complete A-V block and in the clinical management of this lesion. Permanent pacemaker implantation is indicated if complete A-V block persists longer than 2 weeks postoperatively and if the site of the block is within or below the bundle of His.

Supravalvular mitral stenosis associated with tetralogy of Fallot.

The association of tetralogy of Fallot with supravalvular mitral stenosis is a rare anomaly that has been reported only once previously. The difficulty of preoperative diagnosis is emphasized. Although left-sided obstructive lesions in association with tetralogy of Fallot are rare, their recognition is imperative since these are surgically correctable anomalies and potentially lethal, as proved in this case and the one previously reported.

Elective mapping and surgical division of the bundle of Kent in a patient with Ebstein's anomaly who required tricuspid valve replacement.

A 14 year old girl with Ebstein's anomaly and Wolff-Parkinson-White syndrome without supraventricular tachycardia underwent closure of an atrial septal defect and tricuspid valve commissurotomy. Her postoperative course was complicated by her first episodes of recurrent debilitating paroxysmal supraventricular tachycardia. Severe tricuspid insufficiency with low cardiac output necessitated a repeat intracardiac operation. At reoperation the patient underwent successful tricuspid valve replacement with concomitent cardiac mapping and division of the bundle of Kent. In patients with heart disease requiring intracardiac repair who also have Wolff-Parkinson-White syndrome, elective surgical division of the anomalous bundle is recommended whether or not preoperative attacks of tachycardia have occurred.