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PubMed1986

[Monitoring cardiac rhythm after the Mustard intervention for transposition of the great arteries].

Before Mustard's operation 27 children, from 2 days to 11 months old, with simple or complex transposition of the great vessels, underwent an electrocardiogram at rest. After operation, all the children underwent a standard electrocardiogram and 24 hours continuous Holter monitoring. Successive electrocardiograms at rest were recorded in all patients, and eleven children were successfully examined by dynamic electrocardiogram. The electrocardiogram at rest showed a sinusal rhythm in 66.7 of the patients; during the follow-up, in 33.3% non sinusal rhythm was recorded. The continuous 24 hours dynamic electrocardiogram, immediately after surgery, revealed 25.9% of the patients with non-sinusal rhythm. After 7 months, the Holter monitoring showed, in one case, a severe sinus node disfunction. Our data and those of the literature confirm the importance to follow these patients during a long period of time, because it was found that, even bedately, can develop serious arrhythmias.

Electrocardiography↗
PubMed1981

Glenn shunt: long-term results and current role in congenital heart operations.

Fifty cyanotic patients (aged 2 days to 22 years) underwent Glenn shunts for tricuspid atresia and other cyanotic heart defects. Thirteen of 15 operative deaths occurred in infants less than 4 months old, and only 1 death has occurred in the last 9 years. Results were poor in patients with Ebstein's anomaly, truncus arteriosus, transposition of the great vessels, and complex defects other than tricuspid atresia and univentricular heart. Of the 35 patients followed from 0.9 to 14.8 years, 12 were followed for more than 10 years. None of the 11 late deaths could be attributed to complications of the shunt. Minimal evidence of intrapulmonary shunting was found by angiography, pulmonary venous oximetry, or radioisotopic studies. Late deterioration due to venous collaterals and decreased flow to the opposite lung necessitated Blalock-Taussig shunts in 6 and Fontan procedures in 10. All survived the Fontan procedures with minimal morbidity. These data support the concept that Glenn shunts do not necessarily result in pulmonary abnormalities and may be indicated as a staged procedure in a few selected patients prior to a Fontan procedure.

Adolescent↗
PubMed1997

Acquired hydrocephalus associated with superior vena cava syndrome in infants.

We report observations on children with the unusual combination of superior vena cava syndrome in infancy followed by communicating hydrocephalus. Following retrospective review of hospital discharges at a tertiary children's hospital, three children were identified in a 13-year period. Two term infants were treated with extracorporeal membrane oxygenation for pulmonary failure associated with congenital diaphragmatic hernia. These infants had septic complications of central venous lines. A post-term infant required reconstruction of the superior vena cava following cannulation for cardiac bypass surgery to repair transposition of the great vessels. Thrombosis occurred and was followed by the sequential development of superior vena cava syndrome and communicating hydrocephalus. The findings in these patients suggest that communicating hydrocephalus may be caused by superior vena cava syndrome. This is an unusual complication of therapeutic manipulation of the heart and great veins. Cerebrospinal fluid shunting may be required.

Extracorporeal Membrane Oxygenation↗
PubMed1980

A surgical approach for S,L,L single ventricle incorporating total right atrium--pulmonary artery diversion.

Patients with single ventricle and unfavorable anatomy for partitioning procedures have been considered inoperable. Such was the case in two patients with single ventricle and subaortic inverted (hypoplastic) infundibulum associated with subaortic obstruction and levo-transposition of the great vessels (S,L,L). Both had undergone pulmonary artery banding in infancy and presented 11 and 14 years later, cyanotic and severely incapacitated. Both patients were treated surgically by resection of the subaortic obstruction, patch closure of the right artrioventricular valve, closure of the proximal pulmonary artery, and diversion of systemic venous flow through a xenograft valved conduit from the right atrium to the distal pulmonary artery. Early postoperative complications included atrial dysrhythmias and right-sided congestion. The first patient, who had massive cardiomegaly and complete heart block preoperatively, developed congestive failure 20 months postoperatively. The second patient has an excellent functional result and is leading a normal life 1 years after operation. This procedure may be of value for patients with single ventricle in whom corrective ventricular septation is considered too risky or technically unfeasible.

Adolescent↗
PubMed1977

Long-term results of the Mustard procedure.

Over a 10-year period, 123 patients with transposition of the great vessels underwent the Mustard procedure. The hospital mortality for the series was 10.6%. There were seven late deaths, five of which were presumable due to arrhythmia. Sinus rhythm was noted postoperatively in 53% of the patients. Almost all the survivors had achieved catch-up growth when evaluated 6 months to 10 years later. A growth spurt was more consistently seen in the patients who were operated on under 2 years of age. Physical endurance and school performance were on a par with their normal peers or better in about half of the survivors. Severe neurologic problems were present in five patients. Postoperative catheterizations in 42 patients showed a rather high incidence of residual interatrial shunts, mild left ventricular outflow obstruction, and mild superior vena caval obstruction. Pulmonary venous obstruction and serious tricuspid regurgitation were not encountered.

Adolescent↗
PubMed1984

Signal averaged electrocardiography in infants and children with congenital heart disease.

Forty-four patients with congenital cardiac disease underwent surface averaged electrocardiography using a high resolution purpose built module. Twelve patients had complete transposition of the great vessels, 14 had tetralogy of Fallot, 5 had ventricular septal defects, 6 atrial septal defects, 3 atrioventricular septal defects and 4 patients had miscellaneous non-structural abnormalities. All patients with structural disorders underwent corrective or palliative surgery. The aim of the study was to examine the feasibility of the method and its usefulness in detecting His potentials and delayed potentials in the ST segment. A His potential was recorded in 30 patients (68%) with an amplitude ranging from 1.25 to 8.25 microV. Delayed potentials were recorded in only 1 patient with frequent ventricular premature beats. This technique may be useful in assessing intraventricular conduction times in patients with bundle branch block and/or axis deviation especially those who are at risk from intermittent complete AV block after surgical intervention. Detection of delayed potentials may allow better assessment of post-operative risk of ventricular arrhythmias in patients who have undergone ventricular surgery.

Bundle of His↗
PubMed1993

The epidemiology of three serious cardiac defects. A joint study between five centres.

The paper reports a joint study made by five member programs of the International Clearinghouse for Birth Defects Monitoring Systems. Three specific heart malformations were studied, hypoplastic left heart syndrome (HLHS), transposition of the great vessels (TGV), tetralogy of Fallot, and some epidemiological characteristics were analyzed. The prevalence at birth was estimated to be 2.0, 2.9, and 2.2 per 10,000 births, respectively. No time trend in the prevalence at birth was observed for any one of the three malformations in the total study population. When only isolated defects were considered (infants without major non-cardiac malformations), all three cardiac anomalies showed an increased rate in infants with low birth weight, short gestational duration and probably twinning. A preponderance for males was observed for each defect but was strongest among infants with tetralogy of Fallot (sex ratio 2.5 for Fallot, 1.4 to 1.5 for the other conditions). There were also differences between the three cardiac defects with respect to percentage of low birth weight, preterm births, and rate and type of associated extracardiac malformations. This paper stresses the advantage of pooling data from different registries in studies of uncommon specific malformations and infrequent characteristics.

Adult↗
PubMed1990

An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia.

A modified approach to the surgical management of corrected transposition of the great vessels with ventricular septal defect and pulmonary stenosis or atresia was used successfully in two patients. The procedure consisted of performing a venous switch operation, directing the blood flow from the morphologically left ventricle (right-sided chamber) into the aorta through the ventricular septal defect and inserting a valved conduit between the left-sided morphologically right ventricle and the pulmonary artery. This approach has several advantages when compared with the traditional surgical management, which consists of closure of the ventricular septal defect and a left ventricular (right-sided chamber) to pulmonary artery conduit. It uses the morphologically left ventricle as the systemic pumping chamber, thereby minimizing long-term ventricular failure. It allows closure of the defect from the right ventricular side of the septum, thus decreasing the prevalence of complete atrioventricular block. It also avoids use of the tricuspid valve as the systemic atrioventricular valve and therefore decreases the chance of postoperative valve regurgitation.

Adult↗
PubMed1976

Long-time observations on the Blalock-Taussig operation IX. Single ventricle (with apex to the left).

This report concerns a 20 to 28 year follow-up of patients with a single ventricle with pulmonary stenosis and levocardia, a malformation which is frequently combined with a transposition of the great vessels. Only 24 cases were found among the 1037 cyanotic patients with decreased pulmonary blood flow operated on between January 1, 1945 and January 1, 1951. Of the 18 patients who survived surgery, one has been lost to follow-up, eight have lived for more than 20 years, and six for more than 25 years after their initial operation. The results of a Blalock-Taussig operation for a single ventricle are less satisfactory than for a tetralogy of Fallot. Two patients who were living at the close of the study have had no further surgery. Seven patients have had a second operation and four patients have had a third. Progressive cardiac enlargement has been common. The period of enlargement has varied from 10 months to more than 20 years. Three patients have had subacute bacterial endocarditis; two had multiple attacks. One patient died of a brain abscess and one of a purulent meningitis. Three women and four men have married. All four men have A.B. degrees and are gainfully employed.

Adolescent↗
PubMed2000

Transvenous pacemaker insertion ipsilateral to chronic subclavian vein obstruction: an operative technique for children and adults.

Subclavian vein occlusion limits insertion of pacing electrodes in children and adults. The concern is greatest in children with a long-term need for pacing systems necessitating use of the contralateral vein and potential bilateral loss of access in the future. We describe an operative technique to provide ipsilateral access in chronic subclavian vein occlusion in five consecutive pediatric (n = 4, mean age 6.5 years) and adult (n = 1, age 70 with bilateral subclavian vein occlusion) patients in whom this condition was noted at the time of pacemaker or ICD implant. Occlusion was documented by venography. Pediatric cardiac diagnoses included complete heart block in all patients, tetralogy of Fallot in three, and L-transposition of the great vessels in one. Percutaneous brachiocephalic (innominate) or deep subclavian venous access was achieved by a supraclavicular approach using an 18-gauge Deseret angiocath, a Terumo Glidewire, and dilation to permit one or two 9-11 Fr sheaths. Electrode(s) were positioned in the heart and tunneled (pre- or retroclavicularly) to a pre- or retropectoral pocket. Pacemaker and ICD implants were successful in all without any complication of pneumothorax, arterial or nerve injury, or need for transfusion. Inadvertent arterial access did not occur as compared with prior infraclavicular attempts. One preclavicularly tunneled electrode dislodged with extreme exertion and was revised. Ipsilateral transvenous access for pacemaker or ICD is possible via a deep supraclavicular percutaneous approach when the subclavian venous obstruction is discovered at the time of implant. In children, it avoids the use of the contralateral vein that may be needed for future pacing systems in adulthood. This venous approach provides access large enough to allow even dual chamber pacing in children and can be accomplished safely.

Aged↗
PubMed1991

[Experience with a prosthesis of the pulmonary artery trunk at the A.N. Bakulev Institute of Cardiovascular Surgery, Academy of Medical Sciences, USSR].

From April 1975 to March 1991 sixty-four operations were carried out on 61 patients with various congenital heart diseases attended by impaired anatomical connection between the right ventricle and the pulmonary arteries. The patients' ages ranged from 9 months to 28 years (8 years on average). The most frequent clinical diagnoses were total transposition of the great vessels with ventricular septal defect and stenosis of the pulmonary artery (26 cases), type I common arterial trunk (14), Fallot's tetralogy (11 cases). Origin of the aorta and pulmonary artery from the right ventricle with stenosis of the pulmonary artery was encountered in 5 cases, origin of the aorta and pulmonary artery from the left ventricle with stenosis of the pulmonary artery in 4, and type II atresia of the pulmonary artery in one case. Three patients were reoperated on (reimplantation of an artificial pulmonary trunk). Synthetic prostheses with a biological (xenoaortic) valve made at the Institute, Bjerk-Sheily (USA) and Tascon Medical Technology (USA) "conduits", and synthetic prostheses devoid of valves were used. A "fresh" aortic alloprosthesis was used in one case. A statistically significant tendency towards reduction of operative mortality has been recorded (the mortality rate for the last 5 years is 14%). The late-term postoperative results were studied in 30 patients in follow-up periods of 12 months to 14.5 years (5 years and 5 months on average). Analysis of the late-term results shows the method to be highly effective. Stenosis of the prosthesis is the most significant problem of the late-term period.

Adolescent↗
PubMed1975

The pathological anatomy of surgically reconstructable or prosthetically correctable congenital valvular malformation of the mitral region.

The special pathology of reconstructable or only prosthetically correctable congenital malformations of the mitral valve is described on the basis of the following examples taken from our own operative and autopsy material of the last 5 years: 1. Congenital isolated mitral stenosis in female twins (7 month old infant and 33 month old child). 2. Congenital isolated mitral insufficiency in a 7 1/2 year old boy. 3. Combined mixed mitral valve malformations with a parachute valve-like mitral valve anomaly, combined with hypoplasia of the ascending and descending aortas, in a 6 1/2 year old girl. 4. Congenital mitral insufficiency with a parachute mitral valve, combined with supravalvular aortic stenosis and multiple peripheral stenoses of the pulmonary arteries in a 13 1/4 year old boy. 5. Insufficiency of the mitrally inverted tricuspid valve with so-called corrected transposition of the great vessels in a 6 year old boy and with Ebstein's anomaly in a 2 1/2 year old boy. 6. A second mitral ostium in the aortic mitral leaflet with a partial atrioventricular canal in a 6 3/4 year old girl with Ellis-van Creveld syndrome. 7. Bland-White-Garland syndrome with relative mitral insufficiency in a 5 month old and a 4 month old boy. Despite the recurrence of similar and comparable findings, each of our cases of congenital or early acquired noninfectious mitral valve malformation was formally different. n his was also true for the cases of congenital isolated mitral stenosis in twins. Therefore, surgical correction requires a unique procedure for each case. It is possible to reliably infer the degree of malfunction of the atrioventricular valve in a mitral position from the special pathology only by considering the clinical data. On the other hand, a detailed evaluation of congenital mitral valve malformations is possible only through direct inspection--either by the surgeon or through an autopsy--despite modern cardiodiagnostic methods. Typical secondary findings are also discussed--for instance, endocardial fibrosis of the left atrium and the configuration of the heart. The anatomical prerequisites for surgical reconstruction or replacement of the valve with a prosthesis are mentioned.

Adolescent↗
PubMed1988

Myocardial performance after arterial switch operation for transposition of the great arteries with intact ventricular septum.

Left ventricular dimensions, wall thickness, wall stress, and indexes of afterload, preload, contractility, and early diastolic function, as well as regional wall motion, were determined by echocardiographic methods in patients with transposition of the great vessels after arterial switch operation and in age-matched normal controls. In patients evaluated early after surgery, body surface area-adjusted left ventricular dimensions were smaller, and wall thickness was increased compared with controls. Ventricular performance (fractional shortening) was normal in most patients but was abnormally low in 10%. Nevertheless, contractility was normal or augmented in all subjects, with a mean value higher than the control group. The reduction in systolic function was related to altered loading conditions with a combination of reduced afterload and preload combined with augmented contractility. These altered myocardial mechanics appeared to be secondary to routine therapy with digitalis and diuretics. Diastolic function was also normal with differences in the rate of peak filling and rate of wall thinning entirely attributable to differences in ventricular size and function, and normalized indexes of diastolic function were not different between patients and controls. Patients evaluated late after repair were found to have normal regional wall motion with no evidence to suggest regional dysfunction as might be seen with regional ischemia. Ventricular size, wall thickness, systolic function, afterload, preload, contractility, and early diastolic function were indistinguishable from control values. Indexes of diastolic function demonstrated the same relation to age, body surface area, and ventricular size and function in both patients and controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗
PubMed1997

Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience.

OBJECTIVES: Our purpose was to assess the risk factors for late mortality, loss of sinus rhythm and atrial flutter after the Mustard operation. BACKGROUND: The Mustard operation provides correction of cyanosis with low surgical risk in transposition of the great vessels. However, right ventricular failure, loss of sinus rhythm, atrial flutter and death are frequent long-term complications. METHODS: Records of 534 children who underwent the Mustard operation at a single center since 1962 were reviewed for demographic, anatomic, electrocardiographic and physiologic predictors and outcomes. RESULTS: There were 52 early deaths (9.7%). Survival analysis was undertaken for 478 early survivors with a mean follow-up interval of 11.6 +/- 7.2 years. There were 77 late deaths (16.1%), with sudden death (n = 31) the most frequent cause. Survival estimates were 89% at 5 years and 76% at 20 years of age. Risk factors were an earlier date of operation, operative period arrhythmia and an associated ventricular septal defect. Risk (hazard) of late death declined in the first decade, with further peaks in the second decade. Sinus rhythm was present in 77% at 5 years and 40% at 20 years. Loss of sinus rhythm was associated with previous septectomy, postoperative bradycardia and late atrial flutter. Freedom from atrial flutter was 92% at 5 years and 73% at 20 years of age. Risk factors for atrial flutter were the occurrence of perioperative bradyarrhythmia, reoperation and loss of sinus rhythm during follow-up. Risk of atrial flutter demonstrates a late increase. CONCLUSIONS: Ongoing loss of sinus rhythm and late peaks in the risk of atrial flutter and death necessitate continued follow-up.

Arrhythmias, Cardiac↗
PubMed1995

Pulmonary artery banding is not contraindicated in double inlet left ventricle with transposition and aortic arch obstruction.

It has been widely stated that pulmonary artery banding (PAB) is contraindicated in the setting of double inlet left ventricle with transposition of the great vessels (DILV/TGA), especially if aortic arch obstruction is present. We postulated that the poor results for this condition reflect the tendency to leave the band in place long-term without early recognition and relief of subaortic stenosis (SAS). Short-term PAB with early relief of SAS remains an attractive option compared to a neonatal "Norwood" strategy. We reviewed our results applying this approach to 18 consecutive infants presenting since 1980 with DILV/TGA and an obstructive anomaly of the aortic arch (coarctation 16, interruption or atresia 2). Four of the infants (22%) were considered to have important SAS at presentation. One underwent neonatal aortopulmonary connection and died. The remaining 17 patients underwent arch repair with PAB (median age 1.4 weeks; range 2 days-22 weeks) with one early death. The 16 survivors have been followed for 5.6 +/- 3.7 years. All but one ultimately developed SAS. Relief of SAS was performed in 15 patients (median age 8 months) using a proximal aortopulmonary anastomosis. There were two early deaths, and one late death. Thirteen of the 18 patients (72%) are alive and well, and 12 have achieved Fontan repair or bidirectional superior cavopulmonary anastomosis (BCPA) with persistent relief of SAS. Most patients with DILV/TGA and aortic arch obstruction will tolerate temporary PAB with adequate protection of the pulmonary vascular bed. Our current approach (in the absence of severe SAS at presentation) is PAB at the time of arch repair, followed by careful surveillance for, and early relief of, SAS combined with BCPA in infancy.

Anastomosis, Surgical↗
PubMed1998

Ventriculo-arterial discordance: switching the morphologically left ventricle into the systemic circulation after 3 months of age.

OBJECTIVE: To retrospectively examine a 4 year policy of restoring the morphologically left ventricle to the systemic circuit in patients presenting after 3 months of age with ventriculo-arterial discordance with or without associated atrio-ventricular discordance. This policy was stimulated by the known tendency of the morphologically right ventricle to develop dysfunction sooner or later when left in the systemic circuit. Such a policy dictates a more complex surgical approach and, at this point, it remains controversial whether or not the increased surgical complexity is warranted. METHODS: From July 1, 1993 to March 31, 1997, a total of 29 patients were entered into a protocol for placement of the morphologically left ventricle into the systemic circuit. Three groups of patients were identified. Group I; congenitally corrected transposition in 14 patients -- were treated with either a Senning plus arterial switch operation or Senning plus Rastelli procedure. Group II; failed atrial switch procedure in 12 patients of which nine proceeded to arterial switch operation with Senning or Mustard takedown and atrial reseptation. Group III; D-transposition of the great vessels presenting more than 1 year after birth in three patients who underwent arterial switch operation alone. A deconditioned morphologically left ventricle required reconditioning by means of preparatory pulmonary artery banding in 17 of 29 patients. In the patients requiring pulmonary artery banding, an average of 2.1 pulmonary artery bandings was required to prepare the morphologically left ventricle for a systemic pressure workload. RESULTS: In those patients with a deconditioned morphologically left ventricle requiring preparatory pulmonary artery banding, the mean ratio between the left ventricular and right ventricular systolic pressure increased from 0.48 to 0.95. The left ventricular mass increased from 46.6 to 81.8 g/m2 in five patients subjected to serial MRI measurement. Three patients failed the preparatory pulmonary artery banding and did not proceed to anatomical correction. Two subsequently died at a later time. In the patients proceeding to complete anatomical correction: group I -- there were no early or late deaths. Two patients required pacemaker implantation post-operatively. Group II -- there were two in-hospital deaths, one early due to intrapulmonary hemorrhage and one late, secondary to postoperative left ventricular failure with a stormy post-operative course requiring successful ECMO placement and weaning. These patients were 18 and 25 years old, respectively. One patient proceeded to cardiac transplantation 3 months after surgery due to ongoing morphologically left and right ventricular dysfunction. Group III -- all patients continue to do well. CONCLUSIONS: Late anatomic correction of ventriculo-arterial discordance with or without atrio-ventricular discordance can be performed at a relatively low risk. Reconditioning of the morphologically left ventricle can be achieved by sequential pulmonary banding but is not without risk. Failure to achieve adequate reconditioning of the morphologically left ventricle by pulmonary artery banding in the older patient probably increases the risk of non-survival and may be offset by timely transplantation. Longer follow-up and an assessment of the functional status of these patients is required to assess whether or not this complex surgical approach is indeed warranted.

Adult↗
PubMed2006

Great vessel management for endovascular exclusion of aortic arch aneurysms and dissections.

OBJECTIVES: To evaluate a recent approach for the endovascular repair of thoracic aortic aneurysms and dissections involving the aortic arch in high risk patients (HRP). METHODS: Amongst 102 thoracic aortic aneurysms and dissections, we treated 25 patients for aortic arch endovascular exclusion after transposition of the great vessels, of which 14 (56%) had thoracic aortic arch aneurysms and 11 type A and B chronic aortic dissections. Total transpositions were done in 15 cases (60%) and hemi-arch transpositions in 10. We then used Talent, Excluder and Zenith endografts in 12, seven and six cases, respectively. RESULTS: Surgical transpositions were complicated by one minor stroke, which worsened to a major stroke (4%) after endovascular exclusion. After endovascular exclusions, two patients (8%) died from catheterization related complications. One patient had a delayed minor stroke (4%). The successful exclusion rate was 92%. During follow-up (15+/-5.8 months), one patient (4%) developed unilateral limb palsy, successfully treated by CSF drainage. The late exclusion rate remained 92%. No stent-related complications were seen. CONCLUSIONS: Transposition of supra-aortic vessels allows the endovascular exclusion of the aortic arch in HRP. Aortic endografting after surgical transposition proved to be feasible and offers good mid-term results. Specialized surgical centers with both endovascular and surgical expertise are required to treat these patients.

Aged↗