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PubMed2005

Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year experience.

OBJECTIVE: In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. METHODS: The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. RESULTS: The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively. CONCLUSION: Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.

Child↗
PubMed2000

Late complications of tissue glues in aortic surgery.

BACKGROUND: Tissue glues are used in cardiothoracic surgery as an adjunct to operative procedures where tissues are frail, as in aortic dissection, or where added hemostasis is required. This study was undertaken to review the use of tissue glue in our institution over a 5.5-year period. The aim of the study was to identify any potentially glue-related complications. METHODS: A review of tissue glue use for the period from January 1993 to September 1998 was performed and pre-, intra-, and postoperative parameters were collected. After some unusual surgical findings, of special interest was a range of pathology found at late reoperation. RESULTS: A total of 67 cases of tissue glue use were identified, with the majority of operations for type A dissection (76%). There were two intraoperative deaths. Twenty-seven of 65 patients (41%) required 29 further open chest operations; of these, 17 were for acute problems of bleeding or tamponade. Twelve patients (18%) underwent late reoperations months to years later. Nine of these patients, concentrated in two operative groups (7 patients with aortic valve resuspension and 2 patients who had undergone "switch" operations for transposition of great vessels), displayed complications related to the application of gelatin-resorcinol-formaldehyde (GRF) tissue glue. CONCLUSIONS: Indications for tissue glues in cardiothoracic surgery must be carefully considered. We have reviewed our use of some tissue glues in acute type A aortic dissections and in pediatric cardiac patients and have discontinued the use of GRF glues because of unsatisfactory long-term complications.

Aortic Dissection↗
PubMed2006

Feasibility and clinical impact of live three-dimensional echocardiography in the management of congenital heart disease.

BACKGROUND: Precise assessment of congenital heart lesions requires inferential evaluation from multiple two-dimensional echocardiographic images (2DE). The aim of our study was to assess the usefulness of transthoracic live three-dimensional echocardiography (3DE) in the evaluation of congenital heart disease. METHODS: Eighty-two patients (from 4 months to 31 years, mean age 12 +/- 7.5, 38 males and 44 females), known to have congenital heart lesions, prospectively underwent both 2DE and 3DE. Conventional data acquisition by 2DE and "full volume" 3DE acquisition (apical four chambers, parasternal long and short axes, subcostal windows) were carried out by two independent and blinded operators. Data derived from 3DE were compared to 2DE, and 3DE results were graded into three categories: (A) new findings not seen on 2D echo studies, but not critical to therapeutic decision making; (B) additional anatomic information useful in therapeutic decision making; and (C) information equivalent to 2D echo studies. RESULTS: Two out of 82 patients (2%) were excluded because of suboptimal 3DE images. In comparison with 2DE studies, 3DE was graded A in 23 patients (29%), B in 28 patients (35%), and C in 29 patients (36%). In the patients with group B results, atrial and ventricular septal defects, endocardial cushion defects, and l-transposition of great vessels were the most represented pathologies in which 3DE aided medical or surgical therapeutic options. While the new findings in group A did not influence therapy, they defined the whole spectrum of abnormalities in those patients. In patients who fell under group C results, 3DE provided a direct realistic display of the pathology detected by 2DE. CONCLUSIONS: Our study demonstrates that live 3DE, easily performed at the bedside, provides incremental information on patients with a variety of congenital heart lesions. In the clinical scenario, it clarifies the pathology in all its dimensions, particularly in complex lesions with the incremental information having impact on therapeutic decision making.

Adolescent↗
PubMed1979

Cardiovascular anomalies in conjoined thoracopagus twins.

Necropsy findings in 3 cases of conjoined thoracopagus twins are presented. Conjoined hearts were seen lying in a common pericardial cavity. In 2 cases the common atrial complex was incompletely divided into the atrial cavities of the twins by ridges arising from the floor of the cavity. In 1 case the atrial division was better defined and the interatrial septal defect of septum secumdum type was seen. Pulmonary veins opened normally into the atrial chamber only in 1 case, in the other 2 cases, these veins opened into the inferior vena cava and the liver respectively. Ventricular chambers were common in 2 cases with 3 rudimentary atrioventricular openings guarding the inlet and multiple VSD of muscular type. In 1 case one twin had 2 completely separated ventricular cavities and the other twin had partially separated ventricular cavities with a muscular VSD. Great vessels showed transposition in all the cases. In 1 case d-transposition of a rare variety was seen which was associated with a subaortic as well as subpulmonic conus. Persistent ductus arteriosus was a cmmon feature and valvular type of pulmonic stenosis was seen in 2 cases.

Abnormalities, Multiple↗
PubMed1975

Isoproterenol-induced necrotic lesions of embryonic heart tissue.

Chick embryos were used for analyzing the effect of isoproterenol on the embryonic heart. Isoproterenol (2 x 80 mg/kg in 48 hrs) was injected into the amniotic sac from the 5th embryonic day to the time of hatching. From the 5th to the 7th embryonic day (ed), transposition of great vessels occurs. From the 7th to the 14th ed, blockade of development of coronary vascularization was regularly observed, particularly in the ventral wall of the right ventricle. This effect is always associated with persistence of intertrabecular spaces in the nonvascularized area and with a higher water content in the heart. From the 10th to the 12th ed, degenerative changes of the cardiac musculature can be observed. Lesions were characterized by typical myolysis; capillaries in the affected area were dilated and blocked with packed erythrocytes. Necrotic changes were localized only in those parts of the ventricular walls where the vascular bed and myoarchitecture were normally developed. In comparison with similar changes in adult specimens, inflammatory cellularization is lacking.

Animals↗
PubMed1989

[The use of personal computers in the angiocardiometric evaluation of the pulmonary artery in complex congenital heart defects].

Application of personal computers in angiocardiometric assessment of the pulmonary artery in such congenital heart diseases as Fallot's tetralogy and total transposition of great vessels made it possible to set up a convenient data bank allowing one to store numerical and textual information, conventionalized drawings of pulmonary arteries showing the degree and location of stenosing lesions and color-marked data on surgically significant constrictions. The system has a rich visual presentation of data and allows for convenient and quick search and processing of information (including the use of standard statistic, graphic, and other present-day packages of analysis and presentation of medical information).

Adolescent↗
PubMed1989

[Various aspects of the medical treatment of newborn infants with heart disease].

Some aspects of the medical treatment of newborns with congenital heart disease. The outcome of new-born babies with critical congenital heart disease has dramatically improved over the last 25 years. Better understanding of the abnormal hemodynamic situations and thus more appropriate intensive care decisions, increased accuracy of the diagnosis obtained by color-doppler and echocardiography and improved surgical and anesthetic skills and technics, are the main factors of these better results. The authors describe the benefits of prostaglandin infusion in situations where anatomic anomalies prevent the parallel foetal circulation to change into a serial circulation system: severe stenosis or atresia of right or left cardiac valves, transposition of great vessels. Another example of improved medical treatment concerns balloon valvuloplasty. This new technic offers a valuable alternative to surgery in cases of severe isolated stenosis of aortic or pulmonary valves in the neonatal period.

Catheterization↗
PubMed1987

[Prostaglandins in newborn infants with severe cardiopathy].

Between February 1983 and December 1985 authors have studied 26 newborn infants with severe congenital heart disease who received prostaglandin E1 infusion as therapy. Onset of therapy was before 5 days of age in 15 patients, between 5 and 7 days of age in 7 cases and after 14 days of age in the remaining ones. Initial dose was 0.05 mcg/kg/minutes and mean duration of infusion was 131.9 +/- 19.0 hours. Patients were divided into 3 groups according to pulmonary and systemic blood flows: Group I, with cyanosis and diminished pulmonary blood flow (12 cases); Group II, with diminished systemic blood flow (10 cases); and group III, with cyanosis and normal or increased pulmonary blood flow (4 cases). Most patients of groups I (10/12) and II (9/10) improved levels of blood PO2: mean increments of 17.8 +/- 7.4 and 17.5 +/- 13.3 torr respectively. Peripheral pulses also improved in four extremities in patients from group II; systolic and diastolic pressures increased 16 +/- 9.6 and 5 +/- 2.2 mmHg, respectively. No improvement was observed in patients from group III, with exception of patients with transposition of great vessels. Most frequent complication of prostaglandin infusion was hyperthermia, which was observed in 16 cases but disappeared in all with moderate reduction of doses. Appropriate surgery was performed in 19 patients, with an overall survival of 52.6%. In conclusion, prostaglandin infusion appears to be a very valued tool to improve the hemodynamic condition in newborn infants with severe congenital heart disease and thus allow the practice of corrective surgery during first month of life.

Blood Pressure↗
PubMed1985

[Amniotic disease in the mother and severe heart abnormalities in 2 infants].

Detailed clinical presentation including radiographs and palmar dermatoglyphics of one young, healthy woman affected by hand and feet anomalies that at the outset looked alike brachydactyly type B but that are very likely brought about amniotic disease. The problem of genetic counseling came up after the death of two sons born with transposition of great vessels and septal defect but free from malformations of their mother.

Adult↗
PubMed1985

[Congenital heart defects as a cause of still birth and infant mortality].

Among 4,686 still-births and children died from 0 to 1 a in the German Democratic Republic in 1979 there were 307 (6.6%) isolated congenital heart diseases. We only analyzed 294 died infants. The first 5 diagnoses were: Hypoplastic left heart (all infants died within the first 28 d) 9.9%, Ventricular septal defect 9.9%, Transposition of great vessels 8.8%, Coarctation aorta 7.1%, Common truncus arteriosus 5.8%. The most important results are an interesting frequency with advancing years of mothers (3.4% in 10,000 mothers of live-births aged 16 a and less to 20.6% at the age of 40 a and over (see Fig. 1), 13% premature children (under 37 weeks of gestation), 23% children under 2,500 g birth weight, 55% boys and 45% girls. 80% of the above 294 infants died within the first 3 months.

Female↗
PubMedDate not supplied

Multiplane transesophageal echocardiography: our initial experience.

We present our experience in the diagnostic assessment of a wide spectrum of cardiovascular disorders using multiplane transesophageal echocardiography (MP-TEE). Two hundred and seventeen patients in the age range of 11-71 years were subjected to MP-TEE from January to November 1993. The male:female ratio was 1.1:1. One hundred and ten patients had predominantly mitral valve disease of rheumatic origin, eleven had mitral valve prolapse, twenty patients had aortic valve disease and thirty seven patients had more than one valve involvement. Six patients with suspected prosthetic heart valve dysfunction and ten patients of hypertrophic cardiomyopathy were also studied. Two patients had unexplained pulmonary hypertension, three had pericardial disease and three had proximal aortic dissections. Twenty six patients with congenital heart disease were studied of which nineteen had atrial septal defects, one had corrected transposition of great vessels with pulmonic stenosis and one adult had Ebstein's anomaly of the tricuspid valve. In our experience, MP-TEE enhances the versatility of TEE by providing incremental diagnostic information and enhancing delineation of pathology. The procedure was well-tolerated and no complications occurred.

Adolescent↗
PubMedDate not supplied

[A liveborn infant with triploidy (69, XXX): report of one case].

Triploidy is not rare and present in about 1% of all recognized human pregnancies, although most of these pregnancies end in spontaneous abortion during the first trimester. Survival of the fetus up to 20 weeks or beyond is rare. Therefore, liveborn infants with triploidy are very rare. Here is a report on a female liveborn infant with triploidy (69,XXX), who was born to a 27-year-old healthy mother. The clinical features are growth retardation, head-to-body disproportion, wide posterior fontanelle, hypertelorism, micrognathia, bilateral pre-auricular polyps, syndactyly of left 3rd and 4th fingers, syndactyly of right 2nd and 3rd fingers and talipes equinovarus. The infant died 4 hours after birth. The autopsy revealed transposition of great vessels, ventricular septal defect, one lobe of left lung and 2 lobes of right lung and duodenal atresia.

Abnormalities, Multiple↗
PubMedDate not supplied

[Attempt at allo-aortal conduits in the correction of congenital heart defects].

In the period September 1990 to April 1993, an allo-aortic conduit was used in 16 patients during reconstruction of the right ventricular outflow duct. The patients age ranged from 2 years 8 months to 14 years, their body weight was 11.8 to 26 kg. Seven patients were diagnosed as having pulmonary atresia with ventricular septal defect, 4 had Fallot's tetrad and 5 transposition of great vessels with pulmonary stenosis and ventricular septal defect. The allo-aortic conduit made by the procedure developed at the A. N. Bakulev Institute of Cardiovascular Surgery, Russian Academy of Medical Sciences, was used in all cases to reconstruct the right ventricular outflow duct. The allografts were sterilized in a solution containing culture medium RPMI-1640 and mixture of antibiotics. After sterilization, the prostheses were frozen in liquid nitrogen vapours and kept at -150 to -160 degrees C till their clinical application. In the immediate postoperative period, 2 patients died, which amounted to 13%. Two deaths were caused by excessive bleeding, but in one case the implanted conduit wall bled. The residual right ventricle-pulmonary artery gradient was no more than 23 +/- 17 mm Hg. A discharge echocardiographic examination of patients found no valvular dysfunction of the implanted conduits on days 12 to 26 days after surgery. The findings suggest that the allo-aortic conduits prepared at the A. N. Bakulev Institute of Cardiovascular Surgery, Russian Academy of Medical Sciences, can be regarded as the prostheses of choice in the surgery of congenital cardiac malformations.

Adolescent↗
PubMedDate not supplied

[Cryo-treated allo-prosthesis: applications and complications].

From January 1987 to December 1992, fifty two patients aged 1 day to 20 years were implanted cryo-treated homografts into the right-sided position. The patients were diagnosed as having severe Fallot's tetrads, common arterial trunk, transposition of great vessels, vascular bifurcation with pulmonary atresia and isolated pulmonary atresia. The authors implanted 56 homografts, out of them 37 aortic and 19 pulmonary. In the early postoperative period 8 (15.4%) patients died whereas in the late period 1 (1.92%) patient. Out of complications, pseudoaneurysm, calcification with pseudoaneurysm, flap dilatation, and severe prosthesis calcification were found in 1, 1, 1, and 4 patients, respectively. The development of pseudoaneurysms is likely to result from incorrect treatment of donor tissue and to be not so infrequently as previously assumed. To prevent their development is to apply accessory individual sutures to the gaskets in the area of the anterior wall of proximal anastomosis and to use the Gore-Tex material for an external flap.

Adolescent↗
PubMed1997

[Calcified EPTFE vascular prosthesis in the Blalock-Taussig shunt after 4 years of implantation: a case report].

A boy was born with asplenia, complete endocardial cushion defect, pulmonary atresia and transposition of great vessels. Modified Blalock-Taussig (BT) shunt procedure to the right pulmonary artery was performed using an expanded polytetrafluoroethylene (EPTFE) vascular graft three days after born in 1990. At one year old, left BT-shunt procedure was also performed. At four years old (1994), bi-directional Glenn procedure was performed. At that time, a piece of the EPTFE graft in the BT-shunt at the pulmonary anastomosis, was removed and observed macroscopically and microscopically. It was patent with a neointima and thrombi. Microscopically, multiple calcified legions and many macrophages were found in the graft wall. Thrombi on the graft lumen, which were suspected to be formed different occasions, were observed. A thin neointima with an endothelial cell layer near the anastomosis was remarkable. To our knowledge, this is the first report of such calcified lesions in the EPTFE vascular graft wall within 4 years in the BT-shunt position in an infant.

Arteriovenous Shunt, Surgical↗