bluuebunny/crossref_metadata_2025 huggingface dataset:
Mechanisms of injury and methods of protection of the brain during cardiac surgery in neonates and infants
Authors: Greeley William J. , Kern Frank H. , Mault James R. , Skaryak Lynn A. , Ungerleider Ross M.
Published: August 2008

With substantial effort and research devoted to improving surgical techniques and myocardial protection, superb results have been achieved for repair of complex congenital heart defects in children. As a result, investigative efforts now have begun to examine the quality of life for patients surviving these operations. Because these neonates and infants are exposed to severe physiologic extremes of temperature (15–18 °C) and severe alterations from normal perfusion (total circulatory arrest), the nature of long-term neuropsychological outcome has been a prominent concern. Recent preliminary reports suggest that transient and permanent neuropsychologic injury occur in as many as 25% of all infants undergoing hypothermic cardiopulmonary bypass with circulatory arrest.'Since improved surgical techniques have significantly reduced rates of operative mortality and cardiac morbidity, one of the greatest risks remaining for the patient with congenital heart disease may be long term neuropsychologic and developmental abnormalities.
Ultrafiltration and pediatric cardiopulmonary bypass
Authors: Naik Surendra K. , Elliott Martin J.
Published: August 2008

The technique of modified ultrafiltration is a more efficient application of the concept of ultrafiltration during cardiopulmonary bypass. It has been shown to be superior to the conventional method of ultrafiltration.

The method can save considerable quantities of donor blood by returning not only the red cells, but also the white cells, platelets, and clotting factors back to the patient, components which otherwise would be discarded. The elevation of the hematocrit made possible by this method after bypass will permit an “acceptable” hematocrit to be achieved when temperatures become normal after ultrafiltration. The return of platelets and clotting factors along with a high hematocrit will contribute towards significantly minimizing the postoperative loss of blood, and, consequently, the amount of blood requiring to be transfused. The possibility of elevation of the hematocrit allows for a lower hematocrit during bypass. This can achieve a considerable saving of donor blood, particularly in small children who invariably need blood added to the prime, making bloodless pediatric surgery a real possibility. The technique also minimizes the rise in total water in the body after cardiopulmonary bypass in children and promises favorably to alter the course of leaking capillaries as seen frequently in neonates and infants. It has been shown to improve hemodynamics at a crucial time when the patient has been weaned from cardiopulmonary bypass and needs optimum conditions for recovery. From the point of view of research, ultrafiltration offers a window to look at the inflammatory process induced by cardiopulmonary bypass.

It promises to be a valuable technique to investigate the elimination and possibly quantification of toxic metabolites produced during bypass. It also allows evaluation and assessment of different protocols for bypass, involving varying rates of flow and temperature, for their response to the production of these toxic metabolites, thus offering the potential to achieve a model of cardiopulmonary bypass which is as physiological as possible. With advances in the systems of perfusion, aided by computers, it should be possible to control the rate of ultrafiltration and precisely regulate the transfusion of the venous reservoir fluid with the aid of feedback loops from the venous and arterial pressures in the heart. This could make ultrafiltration an integral part of routine cardiopulmonary bypass.
Continuous perfusion without hemodilution in neonates—the experience at the Marie Lannelongue Hospital
Authors: Lacour-Gayet François , Nicolas François , Coil José , Daniel Jean Pierre , Weiss Michel , Pelissie J. , Bruniaux Jacqueline , Planché Claude
Published: August 2008

Cardiopulmonary bypass in neonates is markedly different from the procedure used in children above 10 kg and in adults. There are two major reasons for this—first, the mismatch between the volume of the heart-lung machine circuit and that of the blood in the patient and second, the immaturity of tissues in the first three months of life.
European approach to cardiopulmonary bypass in neonates
Authors: El Habbal Magdi H. , Elliott Martin J.
Published: August 2008

The advent of cardiopulmonary bypass in 1953, and its subsequent continuous refinements, have securely established the specialty of cardiovascular surgery. In conjunction with the improved methods of myocardial preservation, this has allowed the safe repair of acquired and complex congenital cardiac defects. During the years, the many variables of the systems used for cardiopulmonary bypass have been altered to the point that most operations are now reproducible with, relatively, low morbidity and mortality. Although in its present form the system is quite reasonable for most routine surgery, it is far from perfect. There remains, therefore, a quest for alterations to improve the system as an ongoing process. Certain complications of open-heart surgery persist and many new and unique approaches to the concept of organ protection and the treatment of these complications must be developed and refined.
Neonatal cardiopulmonary bypass—a review of current practice in North America
Authors: Groom Robert C. , Hill Aaron G. , Akl Bechara , Kurusz Mark , Lefrak Edward A.
Published: August 2008

One of the most challenging applications of cardiopulmonary bypass is corrective cardiac surgery in the neonate. The small size and high metabolic demand of these patients require miniaturized but efficient equipment. Even with the most advanced components, the volume required to prime the perfusion circuit is typically more than twice the blood volume of a neonate. Neonates have limited cardiac and pulmonary reserves and, therefore, great care is required to preserve those organs that have often already been subjected to hypoxemia, congestive heart failure, or low cardiac output prior to surgery. There is a tendency toward extravascular movement of fluids in newborns subjected to bypass that can adversely affect outcome. Careful monitoring and precise management of perfusion are essential to a successful procedure and optimal recovery of these patients.
Inhibition and restoration of hemostasis in the young cardiac surgical patient
Authors: Jobes David R. , Nicolson Susan C. , Steven James M.
Published: August 2008

For forty years, heparin has been used to temporarily inhibit normal coagulation so as to permit the use of extracorporeal circulation. It has proved effective in preventing gross thrombosis and has not been compromised by changes in adjunctive practices (such as profound hypothermia; circulatory arrest), new pharmacological therapies (such as anesthetics or antibiotics), devices (such as membrane oxygenators), or during use in patients at the extremes of age. Unfortunately, heparin has been and continues to be inefficient in preserving hemostatic function during cardiopulmonary bypass resulting in undesirable bleeding after surgery.
Cerebral perfusion and metabolism after profound hypothermia—comparison between procedures involving no flow and low flow
Authors: Ekroth Rolf , van der Linden Jan , Lincoln Christopher , Scallan Michael
Published: August 2008

The debate concerning no flow versus low flow continues. Thus, it has not yet been possible to conclude whether limited period of total circulatory arrest, as opposed to maintained but reduced systemic flow, offers superior protection of the brain during cardiac surgery in children. While most previous work has focused on the hypothermic period of no versus low flow, less is known about the conditions during and after rewarming with full systemic flow. Some previous data, which related the ischemic marker creatine kinase BB during profound hypothermic procedures, suggested that neural dysfunction was aggravated by posthypothermic factors such as hyperglycemia, acidosis and anemia.
Cerebral cellular response to profound hypothermia
Authors: Watanabe Takao , Washio Masahiko
Published: August 2008

In our first two experiments, we examined brain tissue pH and tensions of oxygen and carbon dioxide in dogs core cooled to 20°C. So as to evaluate the effects of 60 minutes of circulatory arrest, 120 minutes of low-flow perfusion (25 mI/kg/mm), and 120 minutes of moderate-flow perfusion (50 mi/kg/mm), all conducted with and without pulsatile assistance. We further determined the effects of blood gas strategy on the same variables with 60 minutes of circulatory arrest. In a third experiment, we directly observed microcirculation at the surface of the brain during profoundly hypothermic perfusion. In the fourth experiment, we measured cerebral blood flow, oxygen consumption, and excessive production of lactate. Profound anoxia occurred within 20 minutes of circulatory arrest, causing severe and progressive acidosis in the brain tissues along with hypercapnia. The inhalation of 5% or 7% carbon dioxide during core cooling made the brain unacceptably acidotic. The brain acidosis was mild with low flow perfusion, and slight with moderate-flow perfusion. Pulsatile assistance improved acidosis in the brain tissues at all rates of flow. It also improved the microcirculation, the patent number of arterioles and stabilized flow in bridging veins. Cerebral metabolism became aerobic without alterations in cerebral consumption of oxygen during low-flow perfusion. We recommend flow rates above 25% of normal, alpha-stat strategy, and pulsatile assistance for better protection of the brain during profound hypothermia.
Neonatal and infant physiology—impact of cardiopulmonary bypass in the developing patient
Authors: Auten Richard Lambert
Published: August 2008

Cardiopulmonary bypass has been extended to the very young patient undergoing operative correction of congenital heart defects. Growth and development of the central nervous, cardiovascular, pulmonary, and renal systems place significant metabolic and nutritional demands on cellular growth and repair. Immature homeostatic regulation and cellular function require modification of the approaches to preservation of organs and cardiovascular support used in older children and adults undergoing open-heart surgery. Aspects of newborn and infant physiology relevant to cardiopulmonary bypass and postoperative care are reviewed. Current approaches and future strategies designed to address the needs of the developing patient who requires cardiopulmonary bypass are discussed.
Abnormal histology of the aortic arch in coarctation of the aorta
Authors: Wolterbeek Derk W. , Kappetein Arie P. , Gittenberger–de Groot Adriana C.
Published: August 2008

We examined the number of elastic lamellae in the wall of the proximal aortic arch, aortic isthmus and descending aorta in patients with coarctation of the aorta. In the proximal aortic arch, the number of elastic lamellae was significantly lower in patients with coarctation compared to those with normal hearts without aortic anomalies and those with intracardiac defects but without aortic anomalies. The isthmus also showed a significantly lower number of elastic lamellae in the presence of preductal coarctation. In the descending aorta, the number of elastic lamellae was not significantly different between the different groups. There is doubt about the etiology of coarctation. Recent investigations showed that cells from the cardiac neural crest contribute to the formation of the arch arteries and the media of the arch. A developmental error of the neural crest might be responsible for the abnormal mural structures found in patients with aortic coarctation.
Diagnostic utility of color flow Doppler in extracardiac causes of congestive heart failure in infants
Authors: Reed Carl M. , Boulden Thomas F. , Becker Judith A. , Tonkin Ina L. , Alpert Bruce S. , DiSessa Thomas G.
Published: August 2008

Systemic vascular malformations are a rare cause of congestive heart failure in the infant and newborn infant. Clinical diagnosis is often problematic. Accordingly, this study presents the clinical, radiographic, ultrasound imaging, pulsed and color Doppler features of systemic vascular malformations in six infants. In all cases, color Doppler provided vital information regarding size, location, source of feeding arteries and venous drainage essential for appropriate management of these patients. Color flow Doppler ultrasonography is an accurate and sensitive modality for detecting and evaluating vascular malformations.
The pathological diagnosis of pulmonary atresia with abnormal ventriculoarterial connections in the fetus
Authors: Jeffrey Lona Jane Mary , Ho Siew Yen , Moncrieff Deborah Peta Murray , Ross Leslie David , Pearce John Malcolm
Published: August 2008

Pulmonary atresia with abnormal ventriculoarterial connections in the presence of an intact ventricular septum is rare even in postnatal life. Two cases are described in which this combination of lesions was diagnosed in previable fetuses at autopsy. Both pregnancies were terminated for gross structural abnormalities discovered on antenatal ultrasound scans. In the first case, the pulmonary atresia was associated with intact ventricular septum, a double outlet left ventricle and hypoplasia of the tricuspid valve, right ventricle and arterial duct, a combination of lesions that would appear to be unique. In the second case, there was complete transposition and a hypoplastic left ventricle. The severity of the cardiac lesions in both fetuses was such that it is unlikely that either would have survived to term. In the first case no other malformation was present, but in the second there was also a large encephalocoele secondary to early amnion rupture, and a single umbilical artery. The association of the cardiac lesion with early amnion rupture in this second fetus would appear to be a chance event.
‘Malignant’ congenital cardiovascular disease in twins with William's syndrome
Authors: Davis Andrew M. , Burn John , Karl Tom R.
Published: August 2008

Twins of like sex and concordant for William's syndrome are presented. The first twin presented late with aortic arch atresia and has subsequently developed hypertension associated with renal arterial stenosis. The second twin died with myocardial ischemia late after surgery for supravalvar aortic and pulmonary stenosis.
Persistent fifth aortic arch in association with interruption of the aorta proximal to the brachiocephalic artery
Authors: Boothroyd Anne E. , Smith Audrey , Peart Ian
Published: August 2008

We describe a male infant with the combination ofan aortic interruption proximal to the brachiocephalic artery and a persistent fifth aortic arch providing a pulmonary-to-systemic shunt. As far as we know, interruption of the aorta at this site has not previously been reported. Documented cases of a persistent fifth aortic arch itself are few, although failure of recognition may account for its apparent rarity.
Unsuccessful two-stage correction despite appropriate surgery in a case with disconnection (so-called absence) of the right pulmonaty artery
Authors: Gildein Hans P. , von Bernuth Götz , Mocellin Rolf
Published: August 2008

A right-sided modified Blalock-Taussig anastomosis was created using a 5 mm Gore-Tex tube in a 4½ month old infant with unilateral “absence” of the right pulmonary artery. Wide patency of the shunt was confirmed shortly after by angiography, but rarefaction of the peripheral right pulmonary arteries was seen at the age of 2½ years, when corrective surgery was performed. Postoperative hypoperfusion of the right lung, along with severe pulmonary vascular abnormalities seen in a lung biopsy, indicated failure of the two-stage correction in spite of appropriate surgery.
One-stage repair in early infancy of tetralogy of Fallot with ‘absent pulmonary valve’ syndrome
Authors: Yagihara Toshikatsu , Kishimoto Hidefumi , Yamamoto Fumio , Nishigaki Kyoichi , Matsuki Osamu , Uemura Hideki , Takahashi Osahiro , Kamiya Tetsuroh , Kawashima Yasunaru
Published: August 2008

Five infants under three months of age with tetralogy of Fallot and absence of the leaflets of the pulmonary valve were successfully corrected using a one-stage repair. All patients suffered from respiratory failure preoperatively and surgery was performed somewhat urgently. The age at surgery ranged from 26 to 72 days, with an average of 47±17 days. The surgical procedures involved four steps. First, we plicated the aneurysmally dilated central pulmonary arteries. Second, we closed the ventricular septal defect. We then inserted a handmade heterograft pericardial valve at the site of the pulmonary valve, and completed the reconstruction of the right ventricular outflow tract with a patch. All patients survived. Two of them were successfully weaned from ventilation at early (two and 11 days, respectively) periods after surgery, but the remaining three patients required artificial ventilation for one to two months. We conclude that one-stage repair is possible for this anomaly, but that plication of the pulmonary trunk is an essential technique todeliver patients in early infancy from respiratory failure.
Left ventricular function in pulmonary atresia with intact ventricular septum after systemic-to-pulmonary arterial shunt
Authors: Pacileo Giuseppe , Pisacane Carlo , Russo Maria Giovanna , Calabrò Raffaele
Published: August 2008

To investigate the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without coronary-cardiac fistulas after a modified Blalock-Taussig shunt, 14 patients (age range 15 days-16.5 months, mean 4.03±5.6 months) and eight control subjects, matched for age, body surface area and heart rate were evaluated by cross-sectional and Doppler echocardiography. The follow-up interval after palliative procedures ranged from 12 days to 16.3 months (mean 3.67±5.6 months). Compared to controls, in the group of patients the ejection fraction was decreased (61±7% vs 68±5%, p=0.022) while the left ventricular end-diastolic volume indexed for body surface area was increased (72.7±10.8 cc/m246.1±12 cc/m2p=0.0001) with normal values of left ventricular mass indexed for body surface area (67.88±20.9 g/m2vs 76±10 g/m2p=NS). Mass-to-volume ratio was lower in patients with pulmonary atresia (0.95±0.38 vs 1.24±0.3, p=0.031). The left ventricular shape index was increased in all patients with pulmonary atresia (1.27±0.26 vs 1±0.01, p=0.009). A significant inverse correlation was noted between the ejection fraction and follow-up (r=−0.71, p=0.04). as well as between the ejection fraction and shape index (r=−0.76, p=0.048). Moreover, the patients with pulmonary atresia had decreased E/A velocity ratio (0.65±0.16 vs 1.35±0.90, p=0.009), decreased normalized peak filling rate (4.16±0.13 sv/s vs 6.88±0.68 sv/s, p=0.0001), increased peak A velocity (0.95±0.17 m/s vs 0.51±0.16 m/s, p=0.0001) and prolonged isovolumic relaxation time (46±5.4 ms vs 34±6.2 ms, p=0.0001) and deceleration time (196.4±32.2 ms vs 116±21.4 ms, p=0.0001). There was a good correlation between the normalized peak filling rate and follow-up (r=−0.80, p=0.04). These data show a progressive compromise of the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without ventriculocoronary fistulas who had undergone systemic-to-pulmonary arterial shunting. Thus, an earlier biventricular or Fontan type procedure should be recommended.
Frequency and severity of endocardial fibroelastosis in dilated hearts from children—endocardial thickness is inversely correlated to age
Authors: Aiello Vera Demarchi , de Lourdes Higuchi Maria
Published: August 2008

Diffuse endocardial thickening in dilated hearts has been considered as a distinct pathological entity, usually being termed endocardial fibroelastosis. It is common in infants and children and rare in adults. Current opinion holds that the endocardial thickening is a non-specific reaction to stress in the ventricular wall. Our personal observations point to the most severe forms occurring in young infants. To determine the frequency and severity of endocardial fibroelastosis in children with dilated cardiomyopathy, we studied 21 necropsy hearts, both grossly and microscopically. Ages of the patients ranged from two to 175 months (mean 46.5, median 23 months). Using a graduated eyepiece, we measured the endocardial thickness in samples from three sites in the left ventricle and one from the right ventricle. The values were compared to those obtained from similar measurements of the endocardium in hearts from four children presenting with tetralogy of Fallot. All the dilated hearts presented some degree of endocardial thickening. The gross detection of fibroelastosis depended on the degree of thickening. There was a negative but linear correlation between age and the mean endocardial thickness (r=0.70; p>0.001). Our findings support the concept that endocardial cells have greater replicative and secretory potentials in young patients, and that endocardial fibroelastosis is a secondary phenomenon in dilated hearts.
Early and late results of balloon dilation for congenital mitral stenosis
Authors: Alday Luis E. , Juaneda Ernesto , Spillmann Ana , Ruiz Esteban
Published: August 2008

Balloon dilation was attempted in seven symptomatic patients with congenital mitral stenosis aged 7.6±5.3 years. Of the five patients with typical mitral stenosis, four underwent successful dilation with decrease of the mean left atrial pressure and the gradient across the mitral valve and increases in the area of the valve of 127±43 and 85±16%, respectively, as demonstrated hemodynamically or by Doppler echocardiography. In the patient in whom the procedure was unsuccessful, a small child with recurrent pulmonary edema, death occurred during the procedure as a consequence of vasovagal reaction. In the other two patients, one with a parachute-like mitral valve and the other with Shone's syndrome, severe pulmonary hypertension and reversal of the shunt through a patent arterial duct, it was not possible to dilate the valve. In the patient with Shone's syndrome, nonetheless, palliation was achieved by balloon angioplasty of the aortic coarctation and by creation of an atrial septal defect. The patients undergoing dilation remained symptom-free during follow-up and mantained the increase in valvar area as judged by Doppler studies and, in one patient, also by cardiac catheterization. A residual atrial septal defect closed spontaneously in three patients. We conclude that balloon dilation of congenital mitral stenosis is an effective alternative to surgery in patients with typical mitral stenosis. Cross-sectional echocardiography plays an important role in defining valvar morphology and selection of patients. The procedure is not without risk, especially in very sick patients but satisfactory results are long-lasting.
Dissecting aneurysm of the pulmonary arteries—an unusual complication of congenital heart disease
Authors: Farrell Thomas M. , Cottrill Carol M. , O'Connor William N. , Boucher Dede , Noonan Jacqueline A.
Published: August 2008

Dissection of a pulmonary arterial aneurysm due to underlying pulmonary hypertension from congenital heart disease is uniformly fatal, but fortunately rare. Two such cases are presented, along with review of 24 other known cases published in the literature. Clinical presentation, guidelines tomanagement, and possible surgical intervention in the acutely dissecting patient are discussed.
The effect of left-to-right intracardiac shunting on the distribution of pulmonary arterial axial velocities determined by an intraluminal pulsed Doppler technique
Authors: Henry G. William , Criado Enrique , Lucas Carol L. , Ferreiro José I. , Ha Belinda , Carroll Sheri L. , Wilcox Benson R.
Published: August 2008

The distribution of axial velocities in the pulmonary artery must be determined to describe accurately the flow in the pulmonary artery. Previous studies in our laboratory have demonstrated the complexity of the velocity profile in the pulmonary trunk and the branch pulmonary arteries with normal and altered pulmonary blood flow. These findings have demonstrated a skewed, parabolic mean velocity profile in the pulmonary trunk under normal hemodynamic conditions. To determine the effect of left-to-right intracardiac shunting on the distribution of axial velocities in the pulmonary artery, an animal model of atrial and ventricular shunting was used. In both models, the distribution of mean axial velocities remained skewed and parabolic with the higher mean velocities recorded toward the anterior wall.
The morphology of aortopulmonary windows with regard to their classification and morphogenesis
Authors: Ho Siew Yen , Gerlis Leon M. , Anderson Christine , Devine William A. , Smith Audrey
Published: August 2008

We examined 25 heart specimens with aortopulmonary windows in order to review the variety of the lesion and its associated malformations. Unlike common arterial trunk, the aortopulmonary window involves a deficiency of the wall of the ascending aorta and that of the pulmonary trunk. The window was in proximal position in three specimens, intermediate position in three specimens, distal position in 16 specimens and was confluent in three specimens. The size of the window varied from 27 to 100% of the total length of the pulmonary trunk but size did not bear any apparent relationship to the position of the defect. The shape of the window was tunnel-like in one case. Of the 16 specimens with distal windows, the orifice of the right pulmonary artery arose from the aorta in seven specimens and was overriding the plane of the window in one specimen. The window occurred as an isolated lesion in four specimens. It was associated with interruption of the aortic arch at the isthmus in eight specimens and between the left carotid and left subclavian arteries in three specimens. A further three specimens had isthmal stenosis. Among the other associated defects were complete transposition (two specimens), tetralogy of Fallot (one specimen), and double outlet right ventricle (one specimen). A ventricular septal defect was present in six of the 25 specimens. The associated lesions found with aortopulmonary window are seldom encountered with common arterial trunk, suggesting these two arterial lesions have different pathogenesis and are not variants of the same entity.
Magnetic resonance imaging and transesophageal echocardiography—a comparative study in patients with congenital heart disease
Authors: Fletcher Scott E. , Fyfe Derek A. , Wiles Henry B. , McKellar Lisa F.
Published: August 2008

Transesophageal echocardiography and magnetic resonance imaging have proved useful as noninvasive imaging modalities in postoperative patients with congenital heart disease, or patients with suboptimal transthoracic echocardiograms. Each modality has specific advantages. When both are of equal efficacy, magnetic resonance imaging is recommended because it is less invasive and more comfortable for the patient.
Reconstruction of the aortic arch in an adolescent female with complex congenital heart disease
Authors: Verma Rajiv , Danilowicz Delores , Colvin Stephen
Published: August 2008

Coarctations of the aortic arch are rare, and when found, they are often associated with other complex congenital heart disease. An adolescent female with multiple coarctations of the aortic arch and ventricular septal defects underwent complete repair, entailing replacement of the aortic arch with a homograft and closure of the ventricular septal defects. The postoperative course was complicated by a tear in the homograft, possibly related to sepsis. After its resolution, her hemodynamic results remain excellent.
Double orifice mitral valve associated with subaortic stenosis—echocardiographic and anatomic findings
Authors: Johnson Thomas B. , Fyfe Derek A. , Swanger Stephen J.
Published: August 2008

We describe an infant with complex congenital heart disease, including double orifice of the mitral valve associated with complex subaortic stenosis. Transthoracic and intraoperative transesophageal echocardiography were used to define three distinct anatomic abnormalities, all potentially contributing to the subaortic obstruction. Stepwise surgical repair with transesophageal echocardiographic monitoring of hemodynamic function ensured optimal initial surgical results. Overwhelming postoperative complications ensued, however, and the patient died. Postmortem findings are also presented.
Accessory tissue tags arising from the mitral valve—an unusual cause of ventricular flow obstruction
Authors: Knight-Mathis Vicki , Cottrill Carol M. , Salley Robert K.
Published: August 2008

Accessory atrioventricular valvar tissue is uncommon and, on occasion, has been identified as a cause of ventricular outflow obstruction. Accessory tricuspid valvar tissue has been reported to cause subpulmonary obstruction but infrequently has accessory tissue arising from the mitral valve been associated with obstruction. This paper reports two cases of subvalvar obstruction; the first in association with a ventricular septal defect causing subaortic obstruction and the other in association with congenitally corrected transposition and a ventricular septal defect, causing subpulmonary obstruction.
Balloon dilation recanalization of completely occluded modified Blalock-Taussig shunt
Authors: Galal Omar , Qureshi Shakeel A.
Published: August 2008

A two-month-old boy, with complex pulmonary atresia, became increasingly cyanosed 10 days after a modified 5 mm right Blalock-Taussig shunt. There was no shunt flow on auscultation or Doppler echocardiography. Despite anticoagulation with heparin, the shunt remained occluded. After demonstration of the occluded shunt at angiography, a guide wire was easily passed through the occlusion and into the pulmonary artery. Balloon dilation was then successfully accomplished with a 6 mm diameter angioplasty balloon resulting in a patent shunt. The shunt was still widely patent at cardiac catheterization six months later.
‘Absent pulmonary valve’ syndrome associated with muscular ventricular septal defect and aneurysmal dilation of the ascending aorta
Authors: Kececioglu Dennis , Galal Omar , Abbag Fuad
Published: August 2008

A rare association of ‘absent pulmonary valve’ syndrome, midmuscular ventricular septal defect and aneurysmatic dilation of the ascending aorta without pathology of the aortic valve is described in a 10-month-old infant. The diagnosis was established by echocardiography and angiography.
Absence of the arterial duct in a neonate with tricuspid atresia
Authors: Bubolz Beth , Case Christopher L. , Sade Robert M.
Published: August 2008

Summary Absence of the arterial duct has been reported in association with tetralogy of Fallot, with pulmonary stenosis or atresia, other complex cyanotic congenital heart diseases, and anomalies of the aortic arch but not, as far as we are aware, with tricuspid atresia. We report the clinical, surgical, and autopsy findings of a patient with tricuspid atresia whose initial clinical course was influenced by the absence of thearterial duct.
Coronary arterial patterns in complete transposition—;classification in relation to the arterial switch procedure
Authors: Amato Joseph J. , Zelen Jonathan , Bushong Joanne
Published: August 2008

The arterial switch operation has become the standard of surgical repair for total correction of complete transposition (concordant atrioventricular and discordant ventriculoarterial connections). A standard nomenclature for classification of the numerous anatomic relationships the coronary arteries may assume, however, has yet to be universally adopted. Inconsistent use of varied nomenclatures remain an obstacle to effective communication between physicians and surgeons. In this review we offer a unified nomenclature incorporating five important relationships: the configuration of the sinuses; their location; the configuration of the arterial orifices; the pattern of branching of the major coronary arteries; and the course of each artery.
Surgical treatment of ruptured aneurysm of the sinus of Valsalva
Authors: Qiang Guo Jia , Dong Zhu Xiao , Xing Xie Gan , Xiang Cao Jian , Di Xiao Ming , Lin Shen Zhong , Yu Wu Qing , Guo Li Zheng , Wei Shi Cheng , Hua Shang
Published: August 2008

This study reviews the results of the surgical management of 154 cases of ruptured aneurysm of the sinus of Valsalva. Of the patients0 73% were male, with an average age of 28 years. An associated ventricular septal defect was found in 40% and 23% had aortic valvar regurgitation. The aneurysms originated from the right coronary sinus in 79% and from the non-coronary sinus in the remainders. The aneurysms ruptured into the right ventricle in 73%, into the right atrium in 27% and into the left ventricle in less than 1%. Operative mortality was 4.5%. Long-term follow-up was achieved in 80% of patients, with a mean duration of 5.7 years and a range from two months to 29 years. Preoperative aortic regurgitation and preoperative functional class (NYHA III or IV) were both predictive of a worse long-term outcome. The optimal surgical approach was closure of the distal end of the fistula by direct suture together with reinforcement of the aortic sinus with a Dacron patch.
The modified Fontan operation in hearts associated with atrioventricular valvar atresia or common atrioventricular valve—neoseptation of the atriums using a right atrial flap
Authors: Sarioglu Tayyar , Paker Tufan , Türkoglu Halil , Akçevin Atif , Sarioglu Ayse , Yalçinbas Yusuf , Akpinar Belhan , Aytaç Aydin
Published: August 2008

Summary Between June 1988 and December 1992, six patients with dominant left and rudimentary right ventricles underwent orthoterminal correction with a modified Fontan operation in which the atriums were neoseptated using a flap constructed from the right atrial wali. Four patients had anomalous systemic venous connections. The operations were performed under direct caval cannulation, standard cardiopulmonary bypass, moderate hypothermia and cardioplegic arrest. After opening the right atrium with a longitudinal incision, the atrial septum was completely resected and the coronary sinus was cut back. The upper wall of the right atrial incision was brought down in such a way that the pulmonary venous atrium was drained into the dominant ventricle via the right-sided or common atrioventricular valve. This was followed by direct connection of the right atrium with its anterior wall reconstructed with pericardium to the pulmonary arteries. In one patient, a left superior caval vein draining to the left atrium was divided and anastomosed to left pulmonary artery. One patient died on the 12th postoperative day with pulmonary infection and sepsis, though he had no hemodynamic problem, and another died on the sixth day due to high pulmonary vascular resistance. The remaining four patients are progressing well at a mean of 23.4 months postoperatively with functional capacity of NYHA I-IT and sinus rhythm. Echocardiographic and angiocardiographic examinations during follow-up showed unobstructed pulmonary and systemic venous pathways in all. This modification of the Fontan operation seems a good alternative technique which creates a contractile left atrium with large enough dimensions and an unobstructed pathway for pulmonary venous flow.
The role of transesophageal echocardiography in the diagnosis and management of children and young adults with valvar diseases of the left heart
Authors: Leung Maurice P. , Yung Tak-Cheung , Chan Bobby , Chiu Clement , Lee Jan , Mok Che-Kueng
Published: August 2008

The role of transesophageal echocardiography in the diagnosis and management of left-sided cardiac valvar disease in children and young adults was assessed in terms of whether additional information (over transthoracic echocardiography) could be obtained, and whether the added information contributed to the better management of the patients. Between January 1991 to August 1992, 27 consecutive patients were studied. Their age ranged from 2.5 to20 years (mean 12.7), with body weights ranging between 12 and 60 kg (mean 35.6). Twelve of the children suffered from atrioventricular valvar disease, 13 had predominant aortic valvar lesions and two had mixed valvar pathologies. Additional information was obtained by transesophageal over that of transthoracic echocardiography in 18 children (67%). Transesophageal echocardiography clarified the etiologies or mechanism of valvar regurgitation in eight and seven children with atrioventricular and aortic pathologies, respectively. The technique correctly diagnosed severe mitral regurgitation underestimated by conventional technique, detected a missed eccentric aortic regurgitant jet and convincingly excluded bacterial vegetations in one case each. The investigation contributed to the better management of three children (11%) by providing information that allowed the appropriate choice of therapy. Transesophageal echocardiography, therefore, is a useful technique for the diagnosis and management of valvar disease of the left heart in children and young adults.
Absent right atrioventricular connection with the left atrium connected to a left-sided dominant right ventricle—anatomical and functional aspects in four cases
Authors: Ebaid Munir , Cavalini José Fernando , Aiello Vera Demarchi
Published: August 2008

Absent right atrioventricular connection, with the left atrium connected to a dominant left-sided morphologically right ventricle, is a rare situation. We report four cases, demonstrating the anatomical correlations and the clinical aspects needed to make the diagnosis. We also discuss the nomenclature of this important combination.
Cardiac masses in infants and children
Authors: Picarelli Dante , Nozar José Victor , Leone Rubén , Duhagón Pedro , Peluffo Carlos , Montenegro José Luis , Zuñiga Carlos
Published: August 2008

We discuss the management of 10 children with cardiac masses produced bytumors, thrombus or vegetations. The ages of the patients ranged from five days to nine years, only two being more than five years old. In all cases, the diagnosis was made by cross-sectional and Doppler echocardiography. Themasses were detected in the chambers of the right heart in five patients, in the left heart in four and on both sides in one patient. Five patients had predisposing factors. The clinical features were variable, but cardiac failure was the most common sign. All the patients underwent emergency surgery. Two patients died during the period of hospitalization and two in the late postoperative period. The six surviving patients are now asymptomatic.
Placement of endovascular stents for systemic venous obstruction after the Mustard operation
Authors: Abdulhamed Jassim M. , Yousef Saad Al , Ali Khan Mohamed A. , O'Laughlin Martin
Published: August 2008

Three patients aged five years, five years four months and 14 years with obstruction of the systemic venous baffle following the Mustard operation were treated with balloon dilation and implantation ofstents. Balloon dilation of the baffle obstruction was performed initially in the first two cases. In the third case, the obstruction was complete and was punctured with atranseptal needle via a 6 French transeptal sheath followed by a balloon dilation. in all three patients, a Palmaz stent (Johnson & Johnson, Summerville, New Jersey, USA) was loaded onto the balloon catheter and delivered into the stenotic area. There was complete relief of obstruction in allcases. The first case developed supraventricular tachycardia at the time ofcatheterization, the morning following implantation of the stent and thentwo weeks after that. There were no complications with catheterization and noshort-term side effects in the other cases. These cases illustrate the use of endovascular stents in the treatment of baffle obstruction.
Congenital stenosis of pulmonary veins—failure to modify natural history by intraoperative placement of stents
Authors: Cullen Seamus , Ho Siew Yen , Shore Darryl , Lincoln Christopher , Redington Andrew
Published: August 2008

Congenital pulmonary venous stenosis is a rare but important cause of pulmonary hypertension in infancy. The prognosis is uniformly poor, despite surgery and attempted balloon dilation of the narrowed veins. We performed intraoperative stenting of congenital pulmonary venous stenoses in two infants with initial clinical and hemodynamic improvement. Both patients subsequently died due to progressive pulmonary hypertension. Stenting of the pulmonary veins in this condition does not alter the natural history.
Successful surgery for a tuberculous left ventricular aneurysm in a child
Authors: Palweni Chapman W. , Harrisberg Jeffrey R. , Levin Solomon E.
Published: August 2008

An 18-month-old black child became ill one month prior to admission to our center. She was shown to be Mantoux positive, with evidence of cardiomegaly on x-ray. Echocardiography revealed a progressive enlarging left ventricular aneurysm—a finding confirmed at cardiac catheterization. She underwent successful resection of the aneurysm. Histology proved it to be tuberculous in origin. To our knowledge, this is the youngest patient reported with such an aneurysm, and the first to have survived surgical correction.
Double outlet right atrium and complete atrioventricular septal defect with abnormal findings of the biopsied lung
Authors: Suzuki Kiyoshi , Kikuchi Toshio , Mimori Shigekazu
Published: August 2008

We describe a rare type of atrioventricular septal defect with common atrioventricular orifice and malaligned atrial septum, the latter feature resulting in severe obstruction of the left atrial egress. Excision of the atrial septum and banding of the pulmonary trunk was performed at the age of thirteen months. Lung biopsy demonstrated marked medial hypertrophy with severe intimal fibrosis in the small pulmonary arteries and mild thickening of the pulmonary veins, considered contraindications for corrective surgery.
Pitfalls in the diagnosis of pulmonary arteriovenous malformations
Authors: Schiemmer Michael , Tulzer Gerald , Wimmer Maria
Published: August 2008

A homogeneous opacity of the right upper lobe was found radiographically in a 15-month-old male child during investigation of an upper respiratory tract infection. Based on computerized tomography, the diagnosis was made of a solid tumor of the lung. At thoracotomy, dilated serpentine vessels were found on the surface of the right upper lobe, and the diagnosis of a large arteriovenous malformation was considered. Use of enhanced computed tomography without contrast and failure to note a reduced arterial oxygen saturation were two diagnostic pitfalls. The diagnosis of an arteriovenous malformation was confirmed by pulmonary angiography, and a right upper lobectomy was successfully performed.
Intrauterine thrombosis of the arterial duct
Authors: Uzun Orhan , Da Costa Philip E. , Gibbs John L.
Published: August 2008

A hydropic neonate, born at 28 weeks gestation, died 45 minutes after birth. Autopsy showed an abnormally long and tortuous arterial duct which wasoccluded by laminated thrombus. The right heart chambers and the left ventricle were dilated, but there was no other structural cardiac abnormality. Apart from the abnormal ductal morphology, there was no evident cause for the intrauterine thrombosis of the arterial duct, which appeared to be the sole cause of fatal heart failure.
Double chambered left ventricle
Authors: Deshpande Jaya , Sivaraman Ammu , Vaideeswar Pradeep
Published: August 2008

Double chambered left ventricle is a rare anomaly and unlike double chambered right ventricle, is not well documented. We report a case occurring with tetralogy of Fallot, an association not thus far described.
Psychological aspects of congenital heart disease
Authors: Gardner Freda V. , Angelini G. D.
Published: August 2008

Outcome following the treatment of congenital heart disease continues to improve and interest has focused on reducing morbidity as well as mortality. One important aspect of this is the psychological effect of congenital heart disease and its treatment on both children and their parents. This review addresses the extent and nature of the psychological morbidity associated with this disease, in particular the impact of the diagnosis on the management of patient and family. The etiology of the high level of psychosocial morbidity remains poorly understood. Data from the 1960s concerned with pre-school and school-age children is now of little value given the dramatic changes in the treatment of congenital heart disease, which is now predominantly in the neonatal period. While theory from developmental and pediatric psychology may be usefully applied to this area, further research is required to define effective psychological care for patients and their families.
Left ventricular mechanics after early successful repair of aortic coarctation
Authors: Pacileo Giuseppe , Pisacane Carlo , Russo Giovanna M. , Di Donato Roberto M. , Vosa Carlo , Calabrò Raffaele
Published: August 2008

A successful aortic coarctectomy performed beyond early infancy is followed, even in the long term, by persistence of left ventricular hypertrophy and by diastolic dysfunction, although systolic function is often increased. In this study we investigated whether earlier coarctectomy provides better preservation of left ventricular function. Experimental studies on the myocardial response to pressure overload show that neonates and young infants develop a functionally advantageous combination of myocytic hyperplasia (together with mild hypertrophy) and increased angiogenesis. Older patients, in contrast, generate myocytic hypertrophy in isolation, setting the scene for ventricular dysfunction. Cross-sectional echo-Doppler evaluation of left ventricular size, shape, mass and systolic and diastolic function was made in 13 patients a mean of 44±36 months (range 11 days-10 years) after successful coarctectomy in the first year of life. They were compared to 11 age, body surface area and gender-matched control subjects. In all patients, left ventricular mass normalized for body surface area was significantly greater than in the control group (66.2±12.3 vs 43±l2 p=0.0001). There was no correlation between left ventricular mass normalized for body surface area and age at operation, follow-up duration, degree of residual isthmic gradient, peak systolic wall stress, systolic blood pressure or left ventricular shape. No significant differences were noted between the two groups in regard to transverse diameter of the aortic arch, left ventricular afterload (meridional end-systolic wall stress), volume and shape (both in systole and diastole), systolic performance (fractional shortening and ejection fraction) and contractility (rate-corrected velocity of fiber shortening to meridional end-systolic wall stress relationship). Furthermore, no significant differences were found with respect to indices of mitral (including peak filling rate normalized to mitral stroke volume) and pulmonary venous flow, suggesting normal diastolic function. Repair of aortic coarctation in the first year of life promotes a more complete recovery of left ventricular function (particularly diastolic) than that reported after coarctectomy at older age, despite persistence of moderate ventricular hypertrophy.