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Biomedical subjects

E Birk

Publications and source records attributed to E Birk.

At least 19 recordsLinked to original sources
PubMed2001

Thrombus in the left ventricle of a child with systemic emboli: an unusual presentation of hereditary protein C deficiency.

We present a case of left ventricular thrombus in a child with a normal functioning left ventricle. The diagnosis was made by 2-dimensional echocardiography after 2 episodes of systemic emboli. Hereditary protein C deficiency diagnosed in the patient provides the probable pathogenesis of the thrombus formation. Systemic emboli necessitates cardiac examination, and in cases of unusual thrombi, hereditary or acquired thrombophilic risk factors should be considered.

Child, Preschool↗
PubMed2000

Anomalous origin of the left coronary artery from the pulmonary artery: diagnosis and postoperative follow up.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital malformation that presents a diagnostic challenge to the pediatrician and pediatric cardiologist. Although surgical repair is always indicated, the optimal technique has yet to be determined. OBJECTIVES: To review our experience with the diagnosis of children with ALCAPA and to assess short to midterm surgical results. METHODS: Between 1992 and 1998, 13 infants and children (2 months to 15 years) were treated for ALCAPA at our medical center. Eight were diagnosed during the first year of life; all were symptomatic and had severe dysfunction of the left ventricle. The five patients diagnosed at an older age had normal myocardial function. Diagnosis was established by echocardiography alone in seven patients; six required catheterization (one infant and all older patients). Surgery was performed in 12 patients to establish dual coronary artery system: 7 underwent the Takeuchi procedure and 5 had re-implantation of the anomalous left coronary artery. RESULTS: One infant died shortly after diagnosis before surgical repair was attempted, and one died postoperatively. Four patients required additional surgery: three for late complications of the Takeuchi procedure and one valve replacement for mitral insufficiency. Recent evaluation revealed good global left ventricle function in all patients except for one, who is still within the recovery phase and shows gradual improvement. However, most patients who presented with severe myocardial dysfunction upon diagnosis still display abnormal features such as echo-dense papillary muscles or evidence of small akinetic segments. In this group, early repair was associated with faster myocardial recovery. CONCLUSIONS: The diagnosis of ALCAPA remains a clinical challenge to the pediatrician and cardiologist. Diagnosis can be established echocardiographically, and early diagnosis and treatment may lead to faster myocardial recovery. The preferred surgical method appears to be re-implantation of the ALCA. The chance for good recovery of global ventricular function is high even in the sickest patients, nonetheless abnormal myocardial features can be identified even years after surgery.

Adolescent↗
PubMed2000

The Ross operation: initial Israeli experience.

BACKGROUND: The need for aortic valve replacement in children and young adults poses a special problem to cardiologists and surgeons. Replacing the sick aortic valve with the patient's pulmonary valve as described by Ross has proven to be a good option in this special age group. OBJECTIVE: To review our initial experience in order to assess the short-term results. METHODS: From January 1996 to June 1999, 40 patients (age 8 months to 41 years) underwent aortic valve replacement with pulmonary autograft. Indications for surgery were congenital aortic valve disease in 30 patients, bacterial endocarditis in 5, rheumatic fever in 3, and complex left ventricular outflow tract obstruction in 3. Trans-esophageal echocardiography was performed preoperatively and post-bypass in all patients, and transthoracic echocardiography was done prior to discharge and on follow-up. RESULTS: There was no preoperative or late mortality. All patients remain in functional class I (New York Heart Association) and are free of complications and medication. None showed progression of autograft insufficiency or LVOT obstruction. Homograft insufficiency in the pulmonary position has progressed from mild to moderate in one patient, and three developed mild homograft stenosis. CONCLUSIONS: The Ross procedure can be performed with good results in the young population and is considered an elegant surgical alternative to prosthetic values and homografts.

Adolescent↗
PubMed1999

The myocardial profile of the cytosolic isozymes of creatine kinase is apparently not related to cyanosis in congenital heart disease.

BACKGROUND: CKMB, the cardiac-specific heterodimer of cytosolic creatine-kinase (CK), is developmentally and physiologically regulated, tissue hypoxia being a proposed regulator. In patients with cyanotic heart disease the myocardium is perfused with partially saturated blood. We questioned whether the myocardium of cyanotic subjects contains higher proportions of CKMB. MATERIALS AND METHODS: CK activity, the distribution of cytosolic CK isozymes, activity of lactic dehydrogenase (LDH), and tissue protein content were determined in obstructive tissues removed at corrective surgery of patients with congenital heart defects. Cyanotic (n = 13) and acyanotic (n = 12) subjects were compared. RESULTS: In cyanotic and acyanotic patients, CK activity was 8.4 +/- 0.6 and 7.6 +/- 0.6 IU/mg protein and the proportion of CKMB was 21 +/- 1.4 and 22 +/- 2. 0% (mean +/- S.E.M), respectively. In the two groups of patients, the activity related to the B subunit corresponded to the steady-state level of the CKBmRNA. The tissue content of protein and the activities of CK and LDH were similar in cyanotic and acyanotic subjects and increased with the age. CONCLUSIONS: The lack of difference in CKMB distribution between the cyanotic and acyanotic patients may either indicate that hypooxygenation is not a regulator of CK isozyme expression, or may be attributed to the already high proportion of this isozyme in hypertrophied, obstructive tissues. Recruitment of additional CKMB, in the cyanotic hearts, may thus not be required.

Adolescent↗
PubMed1998

[First year's experience of the Post-Operative Cardiac Care Unit, Schneider Children's Medical Center].

In the past 10 years there has been a growing preference for early, complete correction of congenital heart disease. The first year of operation of this cardiac unit is described. 216 operations were performed: 15% in the neonatal age group and 35% in the newborn to 1-year-old groups; 2% were palliative procedures. Mortality was 4.9%. Average stay in the ICU was 3.2 days, with a median of 2.25. Average length of ventilation was 35 hours, with a median of 17.5. Complications were: diaphragm paralysis in 13 (6%), 2/3 of which were recurrent operations; in 2 patients (0.9%) we had to plicate the diaphragm. There was severe neurological damage in 2 which deteriorated to brain death in 1. There was peripheral, reversible neurological damage in 4 (1.8%), and acute renal failure in 3%, with half of them requiring dialysis. 75% of these children died and there was superficial infection in 4.1%, deep wound infection in 1.3%, bacteremia in 4.1%, superior vena cava syndrome in 3 (1.3%) and chylothorax in 2 of them (0.9%). 1 patient (0.45%) required a ventricle-peritoneal shunt after acute viral meningitis. We are encouraged by our results to offer early complete correction to all children with congenital heart disease.

Child↗
PubMed1998

[Transcatheter closure of atrial septal defects: initial clinical applications].

The incidence of congenital heart defects is 0.8% of liveborn infants. Of these 13% are ostium secundum atrial septal defects (ASD) which can be successfully repaired by open heart surgery with less than 1% mortality. However, morbidity associated with cardiac surgery is universal. Transcatheter closure of ASDs was introduced 2 decades ago to decrease surgical complications, hospital stay and cost. Few devices have undergone clinical trials but all of them have been associated with instances of failure and complications. The most important mechanism for acute failure was selection of cases with too large a defect or selection of a defective device. In the past decade great progress has been made with the aid of transesophageal echocardiography (TEE) and modifications of the device. We present our experience in our first 5 patients. They ranged in age from 4 to 27 years; the ASD diameter was 11-15 mm and the device diameter was 17-33 mm. All transcatheter closures were performed on the same day without any complications, and all patients were discharged home after 2 days of hospitalization. We conclude that transcatheter occlusion of ASDs up to 15 mm is feasible, relatively safe and effective. This transcatheter method appears to be a viable alternative to surgery for some patients with secundum atrial septal defects.

Adolescent↗
PubMed1996

Autologous monocusp pulmonary valve: preliminary results.

BACKGROUND: There is growing recognition that postoperative pulmonary regurgitation may result in early or late progressive right heart failure. METHOD: A technique for fashioning an autologous monocusp pulmonary valve from the wall of the pulmonary artery was developed. The monocusp valve was fashioned from the anterior wall of the main pulmonary artery, and the remaining defect was filled with autologous pericardium. The procedure was performed in 8 dogs and 5 children. RESULTS: Early follow-up and serial echocardiographic assessment in both dogs and children proved the functionality of this monocusp pulmonary valve. All valves were pliable and demonstrated mild to moderate pulmonary stenosis and insufficiency. CONCLUSIONS: Construction of the autologous monocusp pulmonary valve is a feasible technique, and the valve performs efficiently. The acute performance in the canine model was excellent, and preliminary midterm results in the clinical study are reasonable. It is logical to assume that the monocusp, being an integral part of the arterial wall, will retain its viability and share in the subsequent growth of the pulmonary artery. Should follow-up studies demonstrate its long-term competence, this autologous valve may provide a good solution for various forms of pulmonary regurgitation and be useful in pulmonary autograft replacement of the aortic valve.

Animals↗
PubMed1996

A one-way valved atrial septal patch: a new surgical technique and its clinical application.

Patients who undergo surgical repair of congenital heart defects, characterized by a hypoplastic right ventricle or high pulmonary vascular resistance, are at high risk for the development of postoperative right heart failure. This risk may discourage the surgical team from carrying out a biventricular or complete repair in such patients. To reduce the risk for right heart failure, we developed a one-way, valved, atrial septal patch to serve as an artificial one-way foramen ovale and tested it in an animal model. By permitting right-to-left shunt, this device decompresses the failing right ventricle and maintains systemic cardiac output. The device has been used in 15 patients divided into three different groups: group 1 (n = 8), patients with a hypoplastic right ventricle and pulmonic stenosis or atresia, seven of whom underwent a biventricular repair; group 2 (n = 5), patients with evidence of pulmonary disease after longstanding left-to-right shunt caused by a correctable atrial or ventricular septal defect, all of whom had a complete repair; group 3, two patients with acute right heart failure in whom the device was used as a last option of treatment to wean them from cardiopulmonary bypass. This article presents our data in regard to the use of the one-way, valved, atrial septal patch and the indications for its clinical use.

Adolescent↗
PubMed1996

Use of the modified technique of ultrafiltration in pediatric open-heart surgery: a prospective study.

The use of cardiopulmonary bypass (CPB) in children is associated with significant morbidity due to the accumulation of an excessive amount of water. This can be decreased by massive diuresis, peritoneal dialysis, or conventional ultrafiltration technique (CUF) during bypass. However, we were dissatisfied with their effect on the outcome of our young patients, and recently began to use the modified technique of ultrafiltration (MUF) with good results. MUF was carried out for 15 min after completion of CPB to a hematocrit of 40%. Eighty patients were equally divided into two groups--MUF (group A) and control (group B)--and prospectively studied. There was one death in group A and two deaths in group B. The chest was left open in one patient in group A and in three patients in group B. At the end of MUF, the cardiac size was smaller, performance was better, and systemic blood pressure was higher in group A. Hematocrit levels in the two groups were similar during preoperative and CPB time, but post-CPB hematocrit in group A was significantly higher, 39.4% mean (range 35-50) vs. 28.5 (range 22-38) in group B (P <0.05). Blood loss was 10.56 ml/kg per 24 h (2-48.7) in group A vs. 20.8 (4.5-105.6) in group B (P <0.05). Blood transfused was 7.2 ml/kg per 24 h (0-29) in group A vs. 17.3 (3.1-49) in group B (P <0.05). Colloids transfused were 16.7 ml/kg per 24 h (0-64) in group A vs. 27.5 (0-58.6) in group B (P <0.05). No significant difference was found in urine output or the use of diuretics between the two groups. MUF results in better hemodynamic status in children, with the added advantage of less transfused blood and blood products. We believe that the use of accepted surgical techniques in combination with MUF will further improve the outcome of complex cardiac malformations.

Blood Loss, Surgical↗
PubMed1995

Surgical management of ventricular septal defect with aortic valve prolapse: clinical considerations and results.

Aortic valve prolapse is found in over 5% of children with ventricular septal defect (VSD). Although this association occurs mostly with doubly committed subarterial VSDs, in this study the predominant type of VSD was perimembranous. In order to determine the need and timing for surgery and whether the anatomical features of septal defect may influence clinical management and outcome in this lesion, we reviewed our experience with 28 consecutive patients, operated on for VSD with prolapsed aortic valve cusp, with or without aortic regurgitation. Twenty-two patients had a perimembranous VSD and six had doubly committed VSD. Aortic regurgitation was trivial or absent in nine patients, mild in ten and moderate to severe in nine. Associated cardiac anomalies were present in 18 patients, all having perimembranous VSD, and included right ventricular outflow tract (RVOT) obstruction (n = 6), discrete subaortic membrane (n = 4) or both (n = 8). None of these patients had more than moderate aortic regurgitation. The patients underwent surgical closure of the septal defect between the ages of 1.5 and 34 years of age (median = 7). Sixteen patients having mild or trivial aortic regurgitation underwent closure of the VSD only, and 12 patients underwent VSD closure with aortic valvuloplasty. Valvuloplasty was required more often in doubly committed VSDs (66%) and in the perimembranous type without associated anomalies (100%), and significantly less often in the presence of RVOT obstruction, subaortic membrane or both (22%). At follow-up (up to 5 years, mean 18 months), the grade of aortic regurgitation was unchanged in 11 and decreased in 5 patients undergoing closure of the VSD only.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1995

Individual anatomy demands various techniques in correction of an anomalous origin of the left coronary artery in the pulmonary artery.

Anomalous origin of the left coronary artery (ALCA) from the pulmonary artery is a rare cardiac anomaly. It can result in decreased myocardial perfusion and impaired left-ventricular function, which can lead to congestive heart failure or even myocardial infarction. Reconstructive surgery of the left coronary artery is the desirable measure in the management of this anomaly. Since July 1992, five patients with ALCA from the pulmonary artery were treated surgically. Age at operation ranged from 3 months to 11 years. Three patients had congestive heart failure and one was in cardiogenic shock. Operative techniques included "tunnel type" surgery in three cases and aortic reimplantation in two. Two operative variations performed successfully in this series are described. There were no postoperative deaths. At the latest follow-up (mean 14 months), all anastomoses were patent and showed antegrade flow. For those patients with ALCA from the pulmonary artery, direct reimplantation of the ALCA to the aorta is the most physiologically appropriate reconstructive solution, and offers good early and late results. Intrapulmonary tunnel from aortopulmonary window to coronary artery is recommended for children in whom aortic reimplantation is not anatomically feasible.

Cardiac Surgical Procedures↗
PubMed1995

Repeated repair of tetralogy of Fallot. Report of 11 cases and review of the literature.

Eleven patients underwent late repeated correction of tetralogy of Fallot in 1991-1993. The previous operation was repair of simple Fallot's tetralogy in seven cases, repair plus transannular patch in one case and repair of tetralogy and pulmonic atresia in three cases. The indications for reoperation were residual ventricular septal defect, right ventricular outflow tract (R.V.O.T.) obstraction, residual branch pulmonary artery stenosis, aneurysmal dilatation of R.V.O.T. Patch or combination of any of the above. At reoperation these defects were corrected. The post operative course was uneventful in eight patients. Two required mechanical ventilation for 2-3 days, and one underwent another operation for residual branch pulmonary artery stenosis. The functional and haemodynamic results were good in ten patients, and one had residual distal pulmonary artery stenosis. There were no death during 2 years of follow-up. Repeated correction of tetralogy of Fallot thus had low postoperative morbidity and good haemodynamic results. For the relatively few patients initially found to have tetralogy of Fallot and pulmonic atresia, the outcome may be less favorable.

Anastomosis, Surgical↗
PubMed1995

Definitive repair of tetralogy of fallot. A review of 104 cases.

Definitive repair of tetralogy of Fallot was performed on 104 children, including 42 younger than 2 years (24 < 1 year and 7 < 6 months). Twenty-two had previous shunt. Transannular patching was required in 60 cases and conduit grafts in two. During 60-day postoperative observation, five patients died. Of the 99 survivors, 93 recovered without complications, three required prolonged mechanical ventilation, two reoperation and one balloon dilatation for residual left pulmonary artery stenosis. Morbidity and mortality were not significantly higher in the patients younger than 12 months, although transannular patching was more common in that age group. Previous shunt operations entailed higher prevalence of distal stenosis and distortion of the pulmonary arteries, which often necessitated surgical repair, and also considerably increased postoperative morbidity and mortality. As the outcome in definitive repair of Fallot's tetralogy is favourable, even in patients younger than 12 months, and as preliminary shunt operations are associated with heightened perioperative morbidity and mortality rates in definitive repair, we advocate the definitive operation for all young patients with severe cyanosis or cyanotic spells.

Adolescent↗
PubMed1995

Interruption of right sided aortic arch. Case report and review of the literature.

Interrupted right sided aortic arch is a very rare anomaly, and to our knowledge only one such case in which the patient underwent complete repair has been recorded in the literature. Twelve additional cases were found on autopsy or underwent palliative surgery. We present the case of a 25-day-old infant with an interrupted right sided aortic arch. The anomaly was repaired by a one-stage surgical approach, and the patient discharged from the hospital. Surgical technique considerations are discussed.

Anastomosis, Surgical↗
PubMed1994

Total cavopulmonary connection (TCPC) for complicated congenital heart malformations.

We reviewed our experience with 40 patients who had undergone total cavopulmonary connection (TCPC) during the past three years. Thirty-one patients had functional single ventricle; only 8 of these with tricuspid atresia, five patients had complex forms of double outlet right ventricle (DORV), and four complex A-V canal. Previous palliative procedures, mostly systemic-pulmonic shunts, were performed in 34 patients. Concomitant procedures were required in 18 patients, mainly reconstruction of distorted pulmonary arteries. A subgroup of 14 high risk patients, that did not fulfil the classical Fontan criteria, underwent 4 mm fenestration of the intra-atrial baffle. There were three (7.5%) early postoperative deaths which occurred in the higher risk group (fenestrated group). However, the remaining patients were all in functional class I or II. Total cavopulmonary connection provides reasonably good definitive palliation for patients with single ventricle physiology. Fenestration of the intra-atrial baffle increases the number of candidates suitable for the Fontan procedure, although the exact inclusion criteria for these patients has yet to be defined.

Adolescent↗
PubMed1994

A one-way, valved, atrial septal patch in the management of postoperative right heart failure. An animal study.

Patients who undergo surgical repair of congenital heart defects that are characterized by hypoplastic right ventricle or pulmonary hypertension are at high risk for the development of postoperative right heart failure. To minimize this risk, a new one-way, valved, atrial septal patch was developed that serves as an artificial one-way foramen ovale. The feasibility of this device was tested in five dogs in which reversible right heart failure had been induced. Use of the one-way valved patch significantly improved right ventricular performance and reduced right ventricular hydrostatic pressures. The interatrial pressure gradient was reduced from 10 +/- 3.5 mm Hg during right heart failure to 4.4 +/- 1.4 mm Hg. When the device was opened, cardiac output increased significantly. This hemodynamic improvement was achieved at the expense of systemic arterial desaturation, which was, however, well tolerated. When the state of right heart failure was reversed, the one-way valved patch spontaneously closed, allowing no interarterial shunting. The results of this experimental trial suggest that this device can play a useful role in selected patients in whom postoperative right heart failure can be anticipated after surgical repair of certain congenital heart defects.

Animals↗