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1.
Pediatr Cardiol ; 26(6): 815-20, 2005.
Article in English | MEDLINE | ID: mdl-16132275

ABSTRACT

The objective of this study was to characterize the transfer of flecainide across the placenta and determine the fetal: maternal ratio of flecainide in the gravid baboon. Flecainide acetate has been especially successful for the treatment of fetal supraventricular tachycardia associated with hydrops fetalis. However, the degree of transplacental transmission remains unknown. In this study, all animals were placed under general anesthesia. Flecainide 2.5 mg/kg was administered intravenously. Percutaneous umbilical blood sampling was performed simultaneously with maternal sampling. Flecainide levels were measured using high-performance liquid chromatography with ultraviolet detection. A total of six gravid baboons were studied at an average gestational age of 132 days. The mean maternal volume of distribution at steady state was 5.1 +/- 1.8 L/kg. The mean combined elimination constant (k(el)) was 0.79 +/- 0.19 hr(-1) [95% confidence interval (CI), 0.64-0.93]. There was a linear relationship between maternal and fetal concentrations, with a ratio of fetal-to-maternal serum levels of 0.49 +/- 0.05 (95% CI, 0.39-0.59). At steady state, fetal flecainide levels are approximately 50% of maternal flecainide levels. Flecainide is rapidly distributed in the mother and fetus following a single intravenous dose with a maternal volume of distribution similar to that reported in normal healthy human adults. Since fetal levels correlate closely with maternal levels, we propose that it is possible to estimate fetal levels by monitoring maternal levels.


Subject(s)
Anti-Arrhythmia Agents/pharmacokinetics , Fetal Blood/metabolism , Fetus/metabolism , Flecainide/pharmacokinetics , Maternal-Fetal Exchange , Animals , Anti-Arrhythmia Agents/administration & dosage , Female , Flecainide/administration & dosage , Hydrops Fetalis/prevention & control , Infusions, Intravenous , Papio , Pregnancy , Tachycardia, Supraventricular/prevention & control
2.
Ann Thorac Surg ; 69(5): 1505-10, 2000 May.
Article in English | MEDLINE | ID: mdl-10881831

ABSTRACT

BACKGROUND: Ebstein's anomaly in the severely symptomatic neonate is usually fatal. Because the mortality for various surgical interventions has been prohibitively high, the indications for operation in these critically ill neonates are unclear. METHODS: We reviewed our results with biventricular repair of three consecutive severely symptomatic neonates (2.8 to 3.2 kg) at our institution since 1994. Each had associated complex cardiac pathology, including multiple muscular ventricular septal defects (n = 1), pulmonary stenosis with functional pulmonary atresia (n = 1), and anatomic pulmonary atresia (n = 1). Preoperatively, all infants had severe tricuspid regurgitation, Great Ormond Street Ebstein echocardiogram scores greater than 1.3:1 (grade 3 or 4) and cardiothoracic ratio greater than 0.85. Two patients were severely cyanotic. Hepatic and renal insufficiency with diffuse coagulopathy was present preoperatively in two patients. Surgical repair consisted of (1) reconstruction of a competent monocuspid tricuspid valve, (2) right ventriculorrhaphy, (3) subtotal closure of atrial septal defect (ASD), (4) aggressive reduction atrioplasty, and (5) repair of all associated cardiac defects. RESULTS: There were no early or late deaths. All patients are currently asymptomatic, without medications, and in sinus rhythm. At 5-year follow-up, trivial tricuspid regurgitation is present in 1 and mild regurgitation in 2 patients. On the basis of these results and review of the current literature, we propose new indications for surgical repair in the neonate with Ebstein's anomaly. CONCLUSIONS: Biventricular repair of Ebstein's anomaly in the critically ill neonate is feasible and medium-term durability of the repair is excellent. Therefore, conventional management of these patients should be revised and early surgical repair encouraged.


Subject(s)
Ebstein Anomaly/surgery , Blood Coagulation Disorders/complications , Ebstein Anomaly/complications , Ebstein Anomaly/diagnostic imaging , Echocardiography , Female , Follow-Up Studies , Heart Septal Defects, Ventricular/complications , Heart Septum/surgery , Heart Ventricles/surgery , Humans , Infant, Newborn , Liver Failure/complications , Male , Methods , Pulmonary Atresia/complications , Pulmonary Valve Stenosis/complications , Renal Insufficiency/complications , Tricuspid Valve/surgery
3.
J Thorac Cardiovasc Surg ; 117(1): 141-6; discussion 46-7, 1999 Jan.
Article in English | MEDLINE | ID: mdl-9869768

ABSTRACT

OBJECTIVE: The purpose of this study was to examine the durability of cryopreserved homografts used to replace the "pulmonary" valve and to identify factors associated with their late deterioration. METHODS: We reviewed our entire experience (1985-1997) with 331 survivors in whom cryopreserved homograft valves (pulmonary, n = 304; aortic, n = 27) were used to reconstruct the pulmonary outflow tract. Median age was 14 years (range, 2 days-62 years). Operations included Ross operation (n = 259), tetralogy of Fallot (n = 41), truncus arteriosus (n = 14), Rastelli operation (n = 11), and others (n = 6). Median follow-up was 3.8 years (range, 0.2-11.2 years); late echographic follow-up was complete for 97% of patients. Homograft failure was defined as the need for explantation and valve-related death; homograft dysfunction was defined as a pulmonary insufficiency grade 3/4 or greater and a transvalvular gradient of 40 mm Hg or greater. RESULTS: Homograft failure occurred in 9% (30 of 331 patients; Kaplan-Meier); freedom from failure was 82% +/- 4% at 8 years. Homograft dysfunction occurred in 12% (39 of 331 patients), although freedom from dysfunction was 76% +/- 4% at 8 years. For aortic homografts, this was 56% +/- 11%, compared to 80% +/- 4% for pulmonary homografts (P =.003). For patients aged less than 3 years (n = 38), this was 51% +/- 12%, compared with 87% +/- 4% for older patients (P =.0001). By multivariable analysis, younger age of homograft donors, non-Ross operation, and later year of operation were associated with homograft failure; younger age of homograft donors, later year of operation, and use of an aortic homograft were associated with homograft dysfunction. CONCLUSIONS: Homograft valves function satisfactorily in the pulmonary position at mid-term follow-up. The pulmonary homograft valve appears to be more durable than the aortic homograft valve in the pulmonary position.


Subject(s)
Aortic Valve/transplantation , Heart Defects, Congenital/surgery , Pulmonary Valve/transplantation , Adolescent , Adult , Child , Child, Preschool , Cryopreservation , Female , Humans , Infant , Infant, Newborn , Male , Middle Aged , Multivariate Analysis , Prosthesis Failure , Pulmonary Valve/surgery , Reoperation , Risk Assessment , Transplantation, Homologous
5.
J Heart Valve Dis ; 6(2): 130-3, 1997 Mar.
Article in English | MEDLINE | ID: mdl-9130119

ABSTRACT

BACKGROUND AND AIMS OF THE STUDY: The Ross procedure involves replacing a transplanted pulmonary valve with a cryopreserved homograft in order to re-establish right ventricle-pulmonary artery continuity. This study reviews mid-term results of such surgery in children and young adults. METHODS: Since November 1986, 114 patients have undergone the Ross procedure at the Children's Hospital of Oklahoma using cryopreserved pulmonary homografts (n = 113) and aortic homograft (n = 1) to reconstruct the right ventricular outflow tract. Graft size ranged from 16 to 32 mm. Ninety-three patients (mean age at implant 10.4 years (range: 0.8-22 years) have had complete mean follow up of 3.2 years (range: 5 months to 8.4 years) after surgery. Homograft evaluation included clinical reports and comparison of early post-implant and latest echocardiography. Measurements of homograft valve annulus and peak instantaneous Doppler gradient were compared; quality of valve leaflets, location of obstruction, and the degree of pulmonary regurgitation were assessed. RESULTS: Compared with early postoperative data, mean homograft annulus size decreased by 15% (p < 0.0001); in 88% of patients, the decrease ranged from one to nine millimeter. Peak Doppler gradient increased significantly (from 10 to 17 mmHg, p < 0.0001); 25% of patients developed gradients > 25 mmHg, and four had gradients > 50 mmHg. Significant obstruction developed most often at the supravalvular level or in the homograft conduit itself. This usually occurred within one year of implant, and was associated with calcification and contracture of the homograft wall. Significant pulmonary regurgitation developed in 19 cases (20%), but was more than mild in only three. Leaflet integrity was maintained except in those who developed severe stenosis or regurgitation. Two patients have undergone re-operation for homograft stenosis 2.8 and 5.4 years respectively after the Ross procedure; one has developed recurrent severe stenosis in the homograft four months later. CONCLUSIONS: After the Ross procedure: (i) Pulmonary homografts undergo significant annular reduction in most patients, though this is usually not associated with the development of significant obstruction. (ii) Peak Doppler gradients across the homograft increase in most patients, though only 4% develop more than mild obstruction. (iii) Mild pulmonary regurgitation is common (20%); moderate or severe regurgitation is rare and usually develops in concert with severe stenosis. (iv) Severe homograft valve degeneration usually occurs within one year of implant, and may reflect an immune-mediated response.


Subject(s)
Aorta/transplantation , Cryopreservation , Heart Defects, Congenital/surgery , Heart Ventricles/surgery , Pulmonary Artery/transplantation , Tissue Transplantation/methods , Adolescent , Adult , Child , Child, Preschool , Echocardiography, Doppler , Evaluation Studies as Topic , Female , Follow-Up Studies , Graft Survival , Heart Defects, Congenital/diagnostic imaging , Heart Ventricles/abnormalities , Humans , Infant , Male , Prognosis , Pulmonary Valve Insufficiency/diagnostic imaging , Pulmonary Valve Insufficiency/etiology , Radiography , Reoperation , Transplantation, Homologous , Ventricular Dysfunction, Right/diagnostic imaging , Ventricular Dysfunction, Right/physiopathology , Ventricular Dysfunction, Right/surgery
6.
Ann Thorac Surg ; 60(6 Suppl): S554-7, 1995 Dec.
Article in English | MEDLINE | ID: mdl-8604934

ABSTRACT

BACKGROUND: We conceptualized that by adding small amounts of prograde pulmonary blood flow to the hemi-Fontan operation, or bidirectional Glenn procedure, this modified hemi-Fontan operation could be safely done at an early age, with better oxygenation, and with less potential for pulmonary arteriovenous fistulae. METHODS: Since April 1992 the hemi-Fontan operation was modified by adding some prograde flow through the native pulmonary artery in 10 high-risk infants, either by leaving the critical subpulmonary stenosis untreated (n = 6) or by tightening a previously placed pulmonary artery band (n = 4). All other sources of pulmonary blood flow were interrupted. Patients were 4 to 23 months old (3.8 to 10.3 kg). Diagnoses included isolated dextrocardia with single ventricle (3) and polysplenia syndrome (2). Cardiopulmonary bypass was needed in 5 patients. RESULTS: There were no hospital deaths. Mean postoperative intensive care unit stay was 2 days, and 9 of 10 patients were discharged within 7 days of operation. One 4-month-old infant with Down's syndrome survived postoperative takedown of the hemi-Fontan repair after pneumonia and caval thrombosis developed. Eight patients are currently asymptomatic receiving minimal modification, and oxygen saturations range from 84% to 93%. CONCLUSIONS: Adding small volumes of prograde pulmonary blood flow to the hemi-Fontan operation is safe, provides improved oxygenation, may encourage growth of central pulmonary arteries, and represent an alternative definitive palliation for high-risk Fontan candidates.


Subject(s)
Heart Bypass, Right/methods , Heart Defects, Congenital/surgery , Palliative Care , Child, Preschool , Humans , Infant , Postoperative Complications , Risk Factors
7.
Ann Thorac Surg ; 59(3): 573-8, 1995 Mar.
Article in English | MEDLINE | ID: mdl-7887692

ABSTRACT

Although closure of ventricular septal defects (VSDs) is currently associated with a relatively low risk, infants with associated atrial septal defects (ASDs) seem to have a higher perioperative morbidity. To clarify this impression, we reviewed our entire experience (since 1977) with closure of simple VSDs in 163 infants (age, < or = 12 months). Of these, 57 had significant ASDs (ASD-VSD subgroup). Hospital mortality was 3.7% (6/163) overall and 1.4% (2/145) since 1980. Actuarial survival at 10 years was 92% +/- 5%. Significant morbidity occurred in 15.5% (16/103) of the VSD subgroup versus 48.1% (26/54) of the ASD-VSD subgroup (p < or = 0.001). Multivariate analysis identified the presence of multiple VSDs and early date of operation as risk factors for hospital death, and younger age, an associated ASD, the size of the VSD, and use of hypothermic circulatory arrest as risk factors for significant perioperative morbidity. Compared with the VSD subgroup, the ASD-VSD subgroup had a higher hospital mortality (5.3% [3/57] versus 2.8% [3/106]), were younger (5.1 +/- 2.9 versus 7.2 +/- 2.9 months; p = 0.001), had a higher preoperative pulmonary artery pressure (70.2 +/- 19.0 versus 62.7 +/- 21.8 mm Hg; p = 0.08), needed more inotropic support (12.3% versus 3.7%; p = 0.07), needed more prolonged ventilation (3.3 versus 1.8 days; p = 0.02), and had longer postoperative hospital stays (11 versus 8 days; p = 0.005).(ABSTRACT TRUNCATED AT 250 WORDS)


Subject(s)
Cardiopulmonary Bypass , Heart Septal Defects, Atrial/complications , Heart Septal Defects, Ventricular/complications , Heart Septal Defects, Ventricular/surgery , Postoperative Complications/epidemiology , Age Factors , Female , Follow-Up Studies , Heart Septal Defects, Atrial/mortality , Heart Septal Defects, Atrial/physiopathology , Heart Septal Defects, Ventricular/mortality , Heart Septal Defects, Ventricular/physiopathology , Hospital Mortality , Humans , Infant , Male , Morbidity , Multivariate Analysis , Postoperative Complications/etiology , Postoperative Complications/physiopathology , Postoperative Complications/surgery , Preoperative Care , Pulmonary Circulation , Reoperation , Risk Factors , Survival Rate
8.
Ann Thorac Surg ; 59(3): 749-51, 1995 Mar.
Article in English | MEDLINE | ID: mdl-7887727

ABSTRACT

A 16-month-old boy suffered a cardiac arrest as a result of acute myocarditis, and venoarterial extracorporeal membrane oxygenation was instituted. Twelve hours later, acute left heart distention developed with cessation of left ventricular ejection. Under transesophageal echocardiographic guidance, a long introducer was placed into the left atrium through a transseptal puncture and connected in-line to the venous circuit. Within hours, left ventricular function improved and ejection returned. Left heart decompression was continued for 5 days, and the patient was weaned from extracorporeal membrane oxygenation after 6 days with normal cardiac and neurologic function.


Subject(s)
Cardiac Catheterization , Extracorporeal Membrane Oxygenation , Heart Arrest/therapy , Heart Atria/pathology , Hypertrophy, Left Ventricular/therapy , Myocarditis/therapy , Punctures , Cardiomegaly/diagnostic imaging , Cardiomegaly/etiology , Cardiomegaly/therapy , Echocardiography, Transesophageal , Heart Arrest/etiology , Heart Arrest/pathology , Heart Arrest/physiopathology , Heart Septum , Humans , Hypertrophy, Left Ventricular/diagnostic imaging , Hypertrophy, Left Ventricular/etiology , Infant , Male , Myocarditis/complications , Myocarditis/pathology , Myocarditis/physiopathology , Severity of Illness Index , Ventricular Function, Left
9.
Ann Thorac Surg ; 57(4): 826-31, 1994 Apr.
Article in English | MEDLINE | ID: mdl-8166526

ABSTRACT

The theoretical advantages of anatomical repair have resulted in the widespread use of the arterial switch operation for transposition of the great arteries. However, preservation of systemic ventricular performance and late functional results have not been well documented. To evaluate late postoperative ventricular function, we reviewed 53 consecutive patients undergoing arterial switch operation for transposition of the great arteries with or without a ventricular septal defect over the 8-year period from March 1985 to 1993. Forty-two patients had simple transposition of the great arteries and 11 patients had associated ventricular septal defects that were closed at operation. Mean age at operation was 1.8 months (range, 1 day to 36 months), and mean patient weight was 3.8 kg (range, 1.8 to 15.6 kg). All but 8 patients were neonates. There were six operative deaths (11.3%, 6/53) and two late deaths during a median follow-up of 23 months (range, 0.1 to 99.5 months). Actuarial survival at 8 years was 83% +/- 6%. Left ventricular outflow tract obstruction has not been identified, and 9 patients (20%, 9/45) have right ventricular outflow tract gradients exceeding 20 mm Hg, 3 of whom have required reoperation. Eighteen patients have mild neo-aortic valve regurgitation. All survivors are currently in New York Heart Association class I, and are in sinus rhythm. Systolic left ventricular function is well preserved with ejection fractions greater than 0.60 in all survivors followed up for more than 4 months (41 patients). Left ventricular end-diastolic volume index is elevated in only 1 patient, a patient who had pulmonary artery banding as a neonate.(ABSTRACT TRUNCATED AT 250 WORDS)


Subject(s)
Abnormalities, Multiple/surgery , Heart Septal Defects, Ventricular/physiopathology , Heart Septal Defects, Ventricular/surgery , Transposition of Great Vessels/physiopathology , Transposition of Great Vessels/surgery , Ventricular Function , Abnormalities, Multiple/mortality , Abnormalities, Multiple/physiopathology , Actuarial Analysis , Aortic Valve Insufficiency/classification , Aortic Valve Insufficiency/diagnosis , Aortic Valve Insufficiency/epidemiology , Aortography , Cardiac Catheterization , Child, Preschool , Confidence Intervals , Echocardiography , Female , Follow-Up Studies , Heart Septal Defects, Ventricular/mortality , Humans , Infant , Infant, Newborn , Male , Postoperative Complications/classification , Postoperative Complications/diagnosis , Postoperative Complications/epidemiology , Proportional Hazards Models , Retrospective Studies , Severity of Illness Index , Stroke Volume , Survival Analysis , Transposition of Great Vessels/mortality , Ventricular Outflow Obstruction/classification , Ventricular Outflow Obstruction/diagnosis , Ventricular Outflow Obstruction/epidemiology
10.
J Card Surg ; 9(2 Suppl): 198-203, 1994 Mar.
Article in English | MEDLINE | ID: mdl-8186567

ABSTRACT

Aortic valve replacement in the child and young adult is often delayed, and multiple operations or invasive procedures are performed to avoid valve replacements. Prosthetic valves, bioprosthetic valves, or allograft valves have been associated with significant complications or early failure and have been a disappointing solution for the patient requiring aortic valve replacement. The pulmonary autograft replacement (PAR) of the aortic valve in children has been shown to be safe and effective with a low incidence of late valve dysfunction. The absence of thromboembolism, the avoidance of anticoagulants, and its viability with the potential for growth and repair strongly support its use for the potential parent, patients of age 35 or less. The experience with 112 patients, 32 females and 80 males, ages 1.5 to 35 years (average 16.1) are reviewed. Twenty-four had aortic insufficiency, 34 had aortic stenosis, and 54 had both aortic stenosis and insufficiency. Actuarial survival was 95.4% +/- 2.0% at 7 years and freedom from reoperation or significant aortic insufficiency of the autograft valve was 92.7% +/- 3.7%. Freedom from all valve related complications of the autograft valve and the homograft replacement of the pulmonary valve was 90.0% +/- 4.0%. Reoperation for the autograft valve was related to limited experience in one, leaflet prolapse and adherence to a VSD patch in one, associated lupus erythematosus in one, and annular and sinotubular dilatation in one. Reoperation of the homograft valve in two patients was secondary to early homograft stenosis, probably due to rejection.(ABSTRACT TRUNCATED AT 250 WORDS)


Subject(s)
Aortic Valve Insufficiency/surgery , Aortic Valve Stenosis/surgery , Aortic Valve/surgery , Pulmonary Valve/transplantation , Actuarial Analysis , Adolescent , Adult , Aortic Valve Insufficiency/etiology , Aortic Valve Stenosis/etiology , Child , Child, Preschool , Echocardiography , Female , Follow-Up Studies , Graft Survival , Humans , Infant , Male , Parents , Pulmonary Valve/diagnostic imaging , Pulmonary Valve/growth & development , Pulmonary Valve/physiopathology , Reoperation , Survival Rate , Transplantation, Autologous/adverse effects , Transplantation, Autologous/methods
11.
Eur J Cardiothorac Surg ; 8(7): 379-83, 1994.
Article in English | MEDLINE | ID: mdl-7946416

ABSTRACT

Pulmonary autograft replacement (PAG) of the aortic valve in children has been shown to be safe and effective with a low incidence of late valve dysfunction. Relief of all types of left ventricular outflow tract obstruction using the pulmonary root has been possible. Concern about the durability of the pulmonary root in the aortic position, and the potential for growth of the pulmonary autograft used either as a root replacement or intraaortic implant, has been questioned. Sixty-five consecutive patients, aged 1.8 to 21 years (mean 12 years) operated on between September 1986 and January 1993, 35 with an intra-aortic implant (IA) and 30 with root replacement (RR), were evaluated by clinical and serial echocardiographic studies (ECHO) up to 6.5 years post-operatively. The hospital mortality rate was 3.0% (70% CL 2.1-5.1%). Two patients required reoperation for PAG insufficiency (AI), one for technical malalignment necessitating replacement at 6 months, and one with progressive leaflet prolapse due to adherence of the valve leaflet to a ventricular septal defect (VSD) patch. Freedom from significant aortic regurgitation at 6-year follow-up was 100% for RR and 91 +/- 6% for IA, and freedom from all valve-related complications including reoperation was 92 +/- 5% at 6 years. Significant enlargement of the aortic annulus which parallels somatic growth has been measured by ECHO in 17 IA implants (P < 0.001) and 17 RR patients (P < 0.01) by 1 year, and in 10 IA (P = 0.007) and 6 RR (P < 0.05) by 2 years.(ABSTRACT TRUNCATED AT 250 WORDS)


Subject(s)
Aortic Valve/surgery , Heart Valve Diseases/surgery , Pulmonary Valve/transplantation , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Infant , Male , Retrospective Studies
12.
Circulation ; 88(5 Pt 2): II198-204, 1993 Nov.
Article in English | MEDLINE | ID: mdl-8222154

ABSTRACT

BACKGROUND: Coarctation repair in the neonate (< or = 28 days) is associated with higher mortality and increased incidence of restenosis compared with older infants. It has been suggested that resection of pericoarctation ductal tissue may reduce this risk of restenosis. METHODS AND RESULTS: To further clarify these issues, we reviewed our experience with 111 consecutive neonates undergoing primary repair between 1973 and 1991. Hospital mortality was 14.4% (16 of 111) and was not significantly different for the type of repair:resection and end-to-end anastomosis (RETE) 10.7% (6 of 56), subclavian flap angioplasty (SFA) 16.7% (6 of 36), and patch angioplasty (PA) 16.7% (3 of 18). Associated complex cardiac pathology was associated with higher operative risk: 25% (10 of 40) versus 8.4% (6 of 71) (P = .02). Median follow-up of 4.2 years (range, 0.1 to 18.5 years) was 99% complete. Late mortality was 13.6% (13 of 95), of which 92% occurred within 1 year of repair. Twenty percent (19 of 95) needed reintervention for restenosis, RETE 16% (8 of 50), SFA 13% (4 of 30), and PA 47% (7 of 15) (P = .02). Of these, 84.2% (16 of 19) required reintervention within 1 year of repair. Freedom from reintervention 1 and 8 years after operation was 80 +/- 4% and 77 +/- 5%, respectively. Actuarial survival 8 years after operation was 73 +/- 4%; for simple coarctation, this was 90 +/- 4%. By multivariate analysis, survival was negatively influenced only by presence of associated cardiac pathology (P = .002) and reintervention only by patch angioplasty technique of repair (P = .007). CONCLUSIONS: In the neonate, resection of coarctation (RETE) does not diminish the risk for reintervention compared with SFA. The risk for both late death and recurrent coarctation are highest within the first year after repair, and follow-up should be particularly vigilant during this period.


Subject(s)
Aortic Coarctation/surgery , Actuarial Analysis , Anastomosis, Surgical/methods , Aortic Coarctation/mortality , Female , Follow-Up Studies , Hospital Mortality , Humans , Infant, Newborn , Male , Multivariate Analysis , Polytetrafluoroethylene , Prostheses and Implants , Recurrence , Retrospective Studies , Risk Factors , Surgical Flaps , Time Factors
13.
Ann Surg ; 216(3): 363-70; discussion 370-1, 1992 Sep.
Article in English | MEDLINE | ID: mdl-1417185

ABSTRACT

Fifty-one children, aged 1.8 to 21 years (mean, 11.4) with aortic valve replacement using a pulmonary autograft are reviewed. Twenty-nine were intra-aortic implants and 22 were root replacements. There was one operative death, no late deaths, and two have required reoperation. Actuarial freedom from reoperation was 93% +/- 5.5 at 5.6 years. Freedom from progression of aortic insufficiency (AI) was 81% +/- 9 at 5.6 years in the intra-aortic implants and 86% +/- 10 in the root replacement. Enlargement of the pulmonary autograft was seen echocardiographically in both groups. This enlargement was consistent with somatic growth and not associated with progression of AI. Ten of 19 patients with aortic stenosis had an LV mass index suggestive of LV hypertrophy before operation. At 1 year, 18 of 25 had a normal LV mass index. Thirteen of 16 patients with AI had preoperative abnormal LV mass index. All but four returned to normal by 1 year. Low operative risk, excellent function, resolution of abnormal LV hemodynamics, and enlargement consistent with somatic growth suggest that the pulmonary autograft is the ideal replacement for the malfunctioning aortic valve.


Subject(s)
Aortic Valve/surgery , Pulmonary Artery/transplantation , Adolescent , Adult , Aortic Valve/diagnostic imaging , Aortic Valve/growth & development , Child , Child, Preschool , Echocardiography , Echocardiography, Doppler , Female , Humans , Infant , Male , Postoperative Complications , Reoperation , Transplantation, Autologous , Treatment Outcome , Ventricular Function, Left
14.
J Card Surg ; 6(4 Suppl): 633-7, 1991 Dec.
Article in English | MEDLINE | ID: mdl-1810559

ABSTRACT

To assess growth potential and hemodynamic sequelae of pulmonary autograft valves implanted into aortic outflow tracts of children, we reviewed our experience with 37 patients (2-21 years) from August 1986 to December 1990. Twenty patients had predominantly aortic stenosis (AS), and 17 had aortic insufficiency (AI). Operative mortality was 3%. Two technical failures required reoperation. Of survivors, six (18%) have moderate AI. Pre- and postoperative echocardiograms were reviewed. The AS group showed increased left ventricular (LV) cavity size by greater than 1-year follow-up, and decreased LV wall and interventricular septal thickness. In the AI group, wall and septal thickness increased by 10 days and LV cavity decreased by 10 days, 60 days, and greater than 1 year. Root replacements (n = 14) showed mean increases of 4.3 mm and 5.3 mm, respectively, in diameters of the aortic annulus and aortic sinuses at greater than 1 year. Intraaortic implants increased 3.1 mm (annulus) and 3.9 mm (sinuses) at greater than 1 year. The pulmonary autograft procedure is safe, and successful implantation normalizes LV dimensions and function rapidly. The autograft valve shows evidence of growth at greater than 1 year postoperative. The pulmonary autograft may be the ideal valve replacement in children.


Subject(s)
Aortic Valve/surgery , Pulmonary Valve/transplantation , Ventricular Function, Left/physiology , Adolescent , Adult , Aortic Valve/diagnostic imaging , Child , Child, Preschool , Echocardiography , Female , Follow-Up Studies , Humans , Male , Postoperative Period , Pulmonary Valve/diagnostic imaging
15.
Ann Thorac Surg ; 50(5): 728-33, 1990 Nov.
Article in English | MEDLINE | ID: mdl-2241332

ABSTRACT

Surgical correction of truncus arteriosus requires the creation of right ventricular to pulmonary artery continuity and closure of the ventricular septal defect. A variety of conduits have been used including valved and nonvalved. Despite a significant incidence of truncal valvar stenosis and insufficiency, this valve has seldom been replaced. We present 4 cases of truncus arteriosus with truncal valvar stenosis or insufficiency that were repaired using two valved homografts: one to create the pulmonary outflow tract and the other to replace the abnormal truncal valve. Two of these patients are doing well after 4 months. Another child survived the operation and did well for 2 months when she died suddenly. The last child died 14 hours postoperatively from low cardiac output syndrome secondary to diabetic hypertrophic cardiomyopathy. When truncal valvar abnormalities are present, the primary repair of truncus arteriosus in an infant should include replacement of the truncal valve. Total correction can be successfully achieved using two valved homografts, resulting in long-term palliation and freedom from thromboembolic events and the use of anticoagulants.


Subject(s)
Abnormalities, Multiple/surgery , Aorta/transplantation , Aortic Valve/transplantation , Heart Valves/abnormalities , Heart Valves/surgery , Truncus Arteriosus, Persistent/surgery , Abnormalities, Multiple/diagnosis , Echocardiography , Female , Heart Septal Defects/diagnosis , Heart Septal Defects/surgery , Humans , Infant, Newborn , Male , Transplantation, Homologous , Truncus Arteriosus, Persistent/diagnosis
16.
Ann Thorac Surg ; 46(5): 579-81, 1988 Nov.
Article in English | MEDLINE | ID: mdl-3190336

ABSTRACT

We report the successful treatment of absent pulmonary valve syndrome in an infant. The treatment consisted of intracardiac repair of pulmonary regurgitation with a homograft valve, which allowed for early extubation and survival.


Subject(s)
Pulmonary Valve/abnormalities , Transplantation, Homologous , Humans , Infant , Male , Pulmonary Artery/surgery , Pulmonary Valve/transplantation , Pulmonary Valve Insufficiency/surgery , Syndrome
17.
Am J Cardiol ; 61(4): 336-40, 1988 Feb 01.
Article in English | MEDLINE | ID: mdl-3341211

ABSTRACT

Adenosine was administered to 25 infants and children (11 patients after presenting with a sustained arrhythmia, and 14 during a diagnostic electrophysiologic study) to determine its electrophysiologic effects. Adenosine was given as an intravenous bolus (starting dose 37.5 micrograms/kg, and increased by 37.5 micrograms/kg increments until an effect was seen). Adenosine caused tachycardia termination or transient increased atrioventricular (AV) block in all 25 patients. Seven patients had tachycardia requiring only the atria for perpetuation and developed increased AV nodal block (minimum effective adenosine dose range 37.5 to 350 micrograms/kg, mean 131). Thirteen had AV reciprocating tachycardia or AV node reentry tachycardia (minimum effective adenosine dose range 37.5 to 225 micrograms/kg, mean 114). Four other patients received adenosine to rule out preexcitation (minimum effective adenosine dose range 37.5 to 375 micrograms/kg, mean 165). One of the 25 patients had junctional ectopic tachycardia and adenosine administration caused retrograde AV block. Six of the 25 (24%) had noticeable but minor side effects. One patient had sustained bradycardia (2 to 3 minutes requiring temporary pacing). Adenosine is a safe and effective agent in the evaluation and treatment of infants and children with arrhythmias.


Subject(s)
Adenosine/therapeutic use , Arrhythmias, Cardiac/drug therapy , Adenosine/administration & dosage , Adenosine/adverse effects , Adolescent , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/physiopathology , Child , Child, Preschool , Electrocardiography , Humans , Infant , Infant, Newborn , Tachycardia/drug therapy , Tachycardia/physiopathology
18.
Circulation ; 76(1): 21-31, 1987 Jul.
Article in English | MEDLINE | ID: mdl-3594769

ABSTRACT

Long RP' supraventricular tachycardias (SVT) often demonstrate both slow and decremental conduction properties in the retrograde pathway of the reentrant circuit. The electrophysiologic properties of these pathways are poorly understood. We studied 10 patients with long RP' SVT (RP'/RR, 0.52 to 0.71); five had the unusual form of atrioventricular nodal reentry (fast-slow) and five patients had accessory AV pathways with slow, decremental retrograde conduction properties. During SVT, the effects of intravenous adenosine (37.5 to 150 micrograms/kg), which increases potassium current (iK) in supraventricular tissue and hyperpolarizes membrane potential toward Ek (-90 mV), and the response to slow-inward channel blockade with verapamil (0.10 to 0.20 mg/kg iv) were evaluated. Adenosine and verapamil has similar effects in the presence of fast-slow AV nodal reentry since both agents terminated SVT by producing block in the retrograde slow AV nodal pathway. In contrast, adenosine and verapamil had differential effects on retrograde conduction in decremental accessory pathways. Adenosine terminated all episodes of SVT in the retrograde decremental pathway, whereas verapamil had a direct effect on this tissue in only two of five patients. Decremental retrograde accessory pathways can therefore demonstrate at least two types of electrophysiologic responses. Pathways that respond only to adenosine-induced hyperpolarizing K+ current likely comprise depressed fast-Na+ channel tissue, i.e., partially depolarized (greater than -60 to -70 mV) atrial tissue. In contrast, decremental accessory pathways that respond to both modulation of the slow-inward calcium current and K+ conductance have pharmacologic properties similar to those of the AV node and may represent more completely depolarized atrial fibers with resting membrane potentials of -60 mV or less.


Subject(s)
Heart Conduction System/physiopathology , Tachycardia/physiopathology , Adenosine , Adolescent , Adult , Aged , Child , Electrophysiology , Humans , Middle Aged , Neural Pathways/physiopathology , Tachycardia, Atrioventricular Nodal Reentry/physiopathology , Verapamil
19.
Am J Cardiol ; 57(15): 1356-62, 1986 Jun 01.
Article in English | MEDLINE | ID: mdl-3717037

ABSTRACT

Transesophageal study was used for diagnosis and treatment of 51 episodes of tachycardia in 13 patients with complete transposition of the great arteries who had undergone atrial baffle procedure. At the time of atrial baffle procedure, patients were 6 to 36 months old (mean 23). Tachycardia (1 to 17 episodes per patient) first occurred 1 to 23 days (4 patients) or 1.8 to 12 years (9 patients) after atrial baffle. Transesophageal study was performed using a bipolar silicone rubber-coated catheter. Tachycardia conversion was accomplished with stimulation bursts using 4 to 10 stimuli 9.9 ms in duration at 20 to 28 mA and an interstimulus interval of 50 to 100 ms less than the atrial cycle length. All tachycardia episodes had regular atrial cycle lengths ranging from 200 to 350 ms. In 12 patients, second-degree atrioventricular (AV) block was observed during tachycardia, suggesting primary atrial tachycardia. However, in 1 patient, occurrence of AV block always resulted in tachycardia termination, suggesting the presence of AV reentrant tachycardia. Transesophageal stimulation converted 48 of 51 tachycardia episodes to sinus/junctional rhythm. Ten tachycardia episodes in 6 patients were transiently converted to atrial fibrillation lasting 3 seconds to 28 minutes before spontaneous conversion to sinus junctional rhythm. Conversion attempts were unsuccessful on 3 occasions. Acceleration of ventricular rate after stimulation necessitated DC cardioversion on 1 occasion. Conversion was not achieved in 2 tachycardia episodes using stimuli less than 10 mA. Transesophageal study is a safe and effective minimally invasive technique for diagnosis and treatment of tachycardia in infants and children who have had atrial baffle for transposition of the great arteries.


Subject(s)
Tachycardia/diagnosis , Child, Preschool , Electrocardiography , Electrophysiology , Female , Heart Atria/surgery , Humans , Infant , Male , Recurrence , Tachycardia/physiopathology , Tachycardia/therapy , Transposition of Great Vessels/surgery
20.
Am J Physiol ; 249(6 Pt 2): H1228-31, 1985 Dec.
Article in English | MEDLINE | ID: mdl-3000199

ABSTRACT

The effects of 0.2 mM octanol on action potential propagation were investigated using in vitro preparations of canine papillary muscles. In these preparations an action potential initiated in the superficial Purkinje (P) layer propagates across specific Purkinje-ventricular junction (PVJ) sites into the underlying ventricular (V) layer. The conduction delay at PVJ sites increased from 4.85 +/- 1.55 to 8.85 +/- 3.34 (mean +/- SD) ms (n = 10, P less than 0.005), an 82% increase. However, propagation within the V syncytium was much less affected, with a decrease of conduction velocity by only 10% and a decrease in the maximal rate of rise of the action potential of 23%. The results indicate that octanol, which has previously been shown to increase gap junctional resistance, has a preferential effect on PVJ sites, as predicted by the hypothesis that there is a restricted pathway for intracellular current flow from P cells to V cells at these sites.


Subject(s)
Heart Conduction System/physiology , Heart/physiology , Octanols/pharmacology , Purkinje Fibers/physiology , Synaptic Transmission/drug effects , 1-Octanol , Action Potentials , Animals , Dogs , Electric Conductivity/drug effects , Heart/drug effects , Kinetics , Purkinje Fibers/drug effects , Time Factors
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