Contributions of Dr. Louis "Pepper" Dehner to the art of cutaneous pathology, the first pediatric dermatopathologist.

Dr. Louis "Pepper" Dehner has been one of the most influential surgical pathologists of the last century. Authoring more than 450 publications, he is the premier modern pediatric pathologist. Perhaps, an area that he is less recognized and in which we would like to describe his contributions, is his role as a creator of the art of pediatric dermatopathology. Dr. Dehner has had at least 50 major publications describing, discovering, and orienting the discipline in the fields of fibrohistiocytic disorders of childhood, vascular tumors, and histiocytosis among many others. Dr. Dehner has clearly manifested that while many similarities between adult and pediatric surgical pathology exist, "children get different diseases." It is because of his mindful analysis and translation of the clinico-pathologic and biologic correlative between specific entities and advances in the field he has made that we are honored to describe some of his contributions to this particular area.

Anomalous left coronary artery from pulmonary artery: autogenous arterial tube for aortic implantation.

Many surgical procedures for the implantation of anomalous left coronary artery from pulmonary artery have been described. A dual coronary system offers most benefit and is most advantageous for the patient. Two autogenous flaps of the pulmonary trunk and ascending aorta, without mobilizing the coronary artery, were created for use in this procedure. This technique was successfully used in an 8 month-old patient with severe myocardial dysfunction and moderate mitral regurgitation. This procedure allows 2 coronary systems to be repaired for any anatomic change of the left coronary artery without the use of prosthetic material. There were no technical complications. During the postoperative course minimal inotropic support was used. The magnetic resonance study, 11 months postoperative, showed wood flow of the left coronary artery and mild mitral regurgitation. The operative technique was simple and its execution easy. The follow-up in the intermediate and late period showed the growth of this endothelized tube.

Conventional and conventional plus modified ultrafiltration during cardiac surgery in high-risk congenital heart disease.

BACKGROUND: This prospective nonrandomized study is the critical assessment of conventional ultrafiltration (CUF) and modified ultrafiltration (MUF) techniques and their efficiency in congenital heart disease surgeries. Use of cardiopulmonary bypass (CPB) in children is associated with body water retention as a consequence of prime volume and systemic inflammatory reaction. The CUF during CPB has reduced body water excess and the MUF after CPB, removes inflammatory mediators, improves hemodynamic performance, and decreases transfusion requirements. METHODS: Forty-one patients, aged 9 to 36 months, submitted to surgical correction for cardiac defects, using CPB, were divided into 2 similar groups: CUF (21 patients) operated between 1996-1997 were ultrafiltered during CPB, and CUF+MUF, (20 patients) operated between 1997-1998 and ultrafiltered during and after CPB. Postoperative duration of ventilator support, pediatric intensive care unit stay (PICU), hospital stay of the groups with and without preoperative pulmonary hypertension (PH), as well as transfusion requirement, hematocrit and platelet counts were compared. RESULTS: There were no technical complications and a significant ultrafiltrate in the CUF+MUF group was observed as compared to the CUF group. No significant differences were observed between the CUF and CUF+MUF groups regarding ventilatory support, PICU stay and hospital stay. Requirements for red cell transfusion, Ht and platelet counts were not statistically different. CONCLUSIONS: CUF and CUF+MUF were safe and efficient methods for patient stabilization independent of diagnosis and complexity of surgery. Future clinical evaluation should address a larger population of patients to research the different variables.

Cytokines and pediatric open heart surgery with cardiopulmonary bypass.

It is well known that, subsequent to cardiopulmonary bypass, and particularly in children, an inflammatory response within the body can often result in a characteristic syndrome. Recently, it has been suggested that this phenomenon is due to a systemic inflammatory response, with significant involvement of cytokines. With this in mind, we investigated the behavior of tumour necrosis factor-alpha and interleukin-6 during the operative and in the immediate postoperative period in a group of children submitted to open heart surgery. We investigated any possible relation between the levels of these cytokines in the serum and the length of cardiopulmonary bypass, with the serum levels of lactate, and with the extent of use of inotropic drugs in postoperative period. The cytokines were measured in samples withdrawn after induction of anesthesia, after 10 minutes of cardiopulmonary bypass, after re-establishment of circulation, and then 2 and 24 hours after the end of cardiopulmonary bypass. The levels of tumour necrosis factor-alpha and interleukin-6 increased between the beginning and at two hours of the end of cardiopulmonary bypass. There was no correlation between the levels of these cytokines in the serum and the length of cardiopulmonary bypass, although there was a positive relation between levels of interleukin-6 and lactate in samples withdrawn at two hours of the end of bypass, and the measured levels of the cytokines correlated with the extent of inotropic drugs employed in the postoperative period.

Assessment of splanchnic perfusion with gastric tonometry in the immediate postoperative period of cardiac surgery in children.

OBJECTIVE - A prospective, nonrandomized clinical study to assess splanchnic perfusion based on intramucosal pH in the postoperative period of cardiac surgery and to check the evolution of patients during hospitalization. METHODS - We studied 10 children, during the immediate postoperative period after elective cardiac surgery. Sequential intramucosal pH measurements were taken, without dobutamine (T0) and with 5mcg/kg/min (T1) and 10 (T2) mcg/kg/min. In the pediatric intensive care unit, intramucosal pH measurements were made on admission and 4, 8, 12, and 24 hours thereafter. RESULTS - The patients had an increase in intramucosal pH values with dobutamine 10mcg/kg/min [7.19+/- 0.09 (T0), 7.16+/-0.13(T1), and 7.32+/-0.16(T2)], (p=0.103). During the hospitalization period, the intramucosal pH values were the following: 7.20+/-0.13 (upon admission), 7.27+/-0.16 (after 4 hours), 7.26+/-0.07 (after 8 hours), 7.32+/-0.12 (after 12 hours), and 7.38+/-0.08 (after 24 hours), (p=0.045). No deaths occurred, and none of the patients developed multiple organ and systems dysfunction. CONCLUSION - An increase in and normalization of intramucosal pH was observed after dobutamine use. Measurement of intramucosal pH is a type of monitoring that is easy to perform and free of complications in children during the postoperative period of cardiac surgery.

Reconstruction of the pulmonary valve and outflow tract with bicuspid prosthesis in tetralogy of Fallot.

BACKGROUND: Right ventricular outflow tract and pulmonary valve implant in patients with tetralogy of Fallot may be required to avoid late postoperative right ventricular impairment. The use of porcine bicuspid prosthesis might be a technical alternative, although comprehensive studies on the long-term use of these prostheses are not available. METHODS: Sixty-three patients (aged 5 months to 34 years; mean, 6 years) with tetralogy of Fallot and pulmonary hypoplasia underwent surgical repair and enlargement of the right ventricular outflow using preserved porcine pulmonary bicuspid prostheses. Fifty-two patients (82.5%) were followed and underwent clinical evaluation and serial Doppler echocardiography. The first 15 patients (29.4%), with ages ranging from 5 to 16 years (mean, 8.2 years) and postoperative follow-up of 48 to 87 months (mean, 65.1 months) underwent hemodynamic and cineangiographic evaluations. RESULTS: There were 11 deaths (17.4%) in the early postoperative period. Of the 52 surviving patients (82.5%), 51 (80.9%) were followed for 1 to 87 months (mean, 42.0 months). Four patients (7.6%) had additional treatment. Of the 15 patients (29.1%) undergoing hemodynamic evaluation, 9 (60%), had mild valvular pulmonary insufficiency and 6 (40%) had moderate insufficiency. Only the right ventricle-to-pulmonary artery pressure gradients and the right ventricular ejection fraction showed statistically significant differences between groups. Right ventricular dimension, although increased in all patients, did not show statistically significant differences. CONCLUSIONS: Right ventricular outflow tract and pulmonary valve repair in patients with tetralogy of Fallot using a bicuspid porcine pulmonary prosthesis is a simple, reliable procedure with good results in postoperative medium term follow-up.

[Anomalous origin of the right pulmonary artery from the ascending aorta (Hemitruncus)]. / Origem Anômala da artéria pulmonar direita em aorta ascendente (Hemitruncus).

The origin of the right pulmonary artery from the ascending aorta is a rare cogenital anomaly, with very few reports in the literature. We describe two cases of this rare malformation, one of them, associated with interruption of the aortic arch (type B). In both cases, the diagnosis was made by two-dimensional echocardiography, with angiographic confirmation in one of them. The origin of the right pulmonary artery was close to the aortic valve, anomaly pathogenetically distinct from the type that arises close to the innominate artery.

[Hemostatic changes in children treated with heart surgery with cardiopulmonary bypass]. / Alterações da hemostasia em crianças submetidas a cirurgia cardíaca com circulação extracorpórea.

PURPOSE: We evaluated hemostasic changes in children undergoing open heart surgery with cardiopulmonary bypass (CPB). METHODS: We studied 17 children before, during surgery, in the immediate, first and between the 4th and 7th postoperative days, measuring hematocrit, prothrombin time, activated partial thromboplastin time, fibrinogen, platelet count, factor V and euglobulin lysis time. Children were divided in those with and without excessive bleeding in the postoperative period. RESULTS: We observed significant prolongation of prothrombin time and activated partial thromboplastin time, reduction of fibrinogen and factor V, and shortening of euglobulin lysis time. Six (35%) children bled excessively. Platelet count reduction was greater in the intra operative period in these cases and the duration of CPB was longer in this group. CONCLUSION: Changes in hemostasis during open heart surgery are due to coagulation cascade disorders as well as fibrinolysis. The incidence of excessive bleeding is higher in the pediatric group. Prolonged CPB time and greater reduction in platelet count differentiated both groups.

Origem anômala da artéria pulmonar direita em aorta ascendente (hemitruncus) / Anomalous origin of right pulmonary artery from ascending aorta (hemitruncus)

A origem da artéria pulmonar direita em aorta ascendente é uma anomalia congênita rara, com poucos casos descritos na literatura. Descrevemos dois casos desta malforamçäo, um associado à interrupçäo de arco aórtico tipo B. Em ambos, o diagnóstico foi realizado mediante ecocardigrafia bidimensional, com confirmaçäo angiográfica em um. A origem da artéria pulmonar direita era próxima à valva aórtica, anomalia patogeneticamente diferente da origem perto da artéria inominada.

Alteraçöes da hemostasia em crianças submentidas a cirurgia cardíaca com circulaçäo extracorpórea / Hemostatic changes in children undergoing heart surgery with bypass

OBJETIVO - Avaliar as alteraçöes de hemostasia encontradas em crianças submentidas a cirurgia cardíaca com circulaçäo extracopórea (CEC). MÉTODOS - Estudamos 17 crianças no pré e pós-operatório (PO) imediato, no 1§ PO e entre o 4§ e 7§ PO, analisando o hematócrito, tempo de protrombina (TP), tempo de tromboplastina parcial ativado, fibrinogênio, contagem de plaquetas, fator V e tempo de lise de euglobulina (TLE). Os pacientes foram divididos em grupos com e sem sangramento execessivo no PO. RESULTADOS - Houve alteraçöes significantes no intraoperatório com aumento do TP e tempo de tromboplastina parcial ativado e reduçäo do fibrinogênio, fator V e do TLE. Seis (35 por cento) crianças sangraram execessivamente. A contagem de plaquetas foi significantemente menor no intra-operatório; neste grupo o tempo de CEC foi menor. CONCLUSÄO - Alteraçöes de hemostasia no intra e PO ocorrem como conseqüência da ativaçÝo da cascata de coagulaçäo e fibrinólise. A incidência de sangramento execessivo é alta nesta faixa etária. Os grupos com e sem sangramento execessivo diferenciam-se pelo maior tempo de CEC e contagem reduzida de plaquetas.

Anomalous origin of the right coronary artery from the pulmonary artery in association with a ventricular septal defect.

Origin of the right coronary artery from the pulmonary artery is a rare lesion occasionally found at angiography or autopsy. We report the rare preoperative diagnosis, in a child, of anomalous origin of the right coronary artery from the pulmonary artery, in association with a ventricular septal defect. The chest radiograph was normal, but auscultation revealed a continuous murmur at the left sternal border and electrocardiography showed right and left ventricular hypertrophy. A transthoracic echocardiogram depicted anomalous origin of the right coronary artery from the pulmonary artery. Color-flow Doppler echocardiography indicated possible right-coronary-artery-to-right-ventricle fistulae. Diagnosis was made by selective left coronary arteriography, which showed retrograde filling of the right coronary artery from collateral vessels. Selective left coronary arteriography depicted intercoronary flow, with no fistulae. Operative repair consisted of moving the proximal right coronary artery from its origin at the pulmonary trunk to the aorta. An associated procedure for correction of the ventricular septal defect was performed. The postoperative cardiac angiogram showed that the ventricular septal defect was closed and that flow through the right coronary artery was normal. Preoperative diagnosis of anomalous origin of the right coronary artery from the pulmonary artery is important, because this condition is surgically correctable.

[Heart surgery in Brazilian Indians]. / Cirurgia cardíaca em índios brasileiros.

PURPOSE: Our experience with surgical treatment of heart diseases in Indians living in the Amazon rain forest in primitive stages was reviewed. METHODS: From 1988 to 1995, 18 patients underwent cardiovascular surgical procedures at the São Paulo Hospital of the Escola Paulista de Medicina. Seven patients had valvar disease, nine congenital heart defects, one submitral aneurysm and one arrhythmia. Thirteen Indians came from tribes of the Amazon rain forest area: three from the Xavante, two from Waiapi, two from Tucano, two from Macuxi, two from Mayoruna, and one of each tribe of Xikrin, Guajajara, Terena, Surui, Galibi, Cinta-Larga and Pataxó. RESULTS: We performed 22 operations, with two hospital deaths. Follow-up was possible in 87.5% of cases, with one late death. The majority of cases were due to congenital heart defects and in this series it was noted the absence of operations to treat coronary artery disease. The incidence of valve disease was higher in accultured or semi-accultured Indians. CONCLUSION: The surgical treatment of cardiovascular disease has made possible to the surviving indians to return to and be accepted by their fellow tribesmen.

Transaortic approach has a role in the surgical treatment of ventricular septal defects.

A transaortic approach was used for surgical closure of ventricular septal defects (VSDs), sometimes as first choice and otherwise as an alternative approach. This paper presents the experience in a group of 124 patients who underwent VSD closure by a transaortic approach in an 18-year-period, between 1974 and 1992. Patients were divided in three groups: (I) Isolated VSD, 89 patients (mean age 7.6 years); (II) VSD associated with other malformations, 27 patients (mean age 10.8 years); and (III) recurrent VSD, eight patients (mean age 15.6 years). Two deaths occurred in the postoperative period (2/124), one after closure of VSD in a patient with severe pulmonary hypertension; another death was observed after transaortic closure of the VSD as part of the repair of a complex heart malformation. There were no instances of new right bundle branch block (RBBB) in the group operated on for isolated VSDs. Three cases of left bundle branch block (LBBB) (3.4%) occurred in the isolated VSD group. There were no cases of complete aortic valve (AV) block in the whole series and no instances of AV dysfunction. The transaortic approach is an alternative for the surgical treatment of ventricular septal defects. The ease of exposure and closure of defects suggest this technique to be a particularly attractive approach in the treatment of small to moderate-sized VSDs and also in recurrent VSDs.

[Anomalous venous drainage of the left lung. Clinical and surgical findings and diagnostic difficulties]. / Drenagem venosa anômala de pulmão esquerdo. Achados clínicos e cirúrgicos e dificuldades diagnósticas.

PURPOSE: To analyze clinical and surgical aspects of patients with anomalous left pulmonary venous drainage (ALPVD). METHODS: Seven patients, 3 males, with ages ranging from 18 months to 29 years were retrospectively studied, by analyzing the symptoms, electrocardiograms, chest X-ray, echocardiograms, angiography and surgical technique. RESULTS: All patients, but one, were symptomatic being the effort dyspnea the most prevalent symptom. Electrocardiogram showed incomplete right bundle branch block in all but one case. Chest X-ray showed prominent pulmonic vessels (6 patients), different degrees of cardiomegaly (6) and a left superior vena cava like image (3). Echocardiography correctly depicted the anomaly in 4 cases. The ALPVD was lobar in 4 cases and total in 3. In 3 patients there was pulmonary hypertension and in one venous drainage obstruction (8mmHg gradient). Six patients were operated on, 3 through a sternotomy approach with cardiopulmonary bypass and other 3 through a left thoracotomy without cardiopulmonary bypass. CONCLUSION: ALPVD has varying clinical repercussion and its clinical picture resembles atrial septal defect. Some cases may present diagnostic difficulties and echocardiograms may result in false negative findings. Cardiac catheterization is important to obtain detailed anatomic diagnosis, to evaluate the degree of pulmonary hypertension and to rule out venous obstruction. Left thoracotomy without extracorporeal circulation is indicated in ALPVD without associated defects.

[Supravalvar aortic stenosis complicated by aortic endarteritis and formation of mycotic aneurysm]. / Estenose aórtica supravalvar complicada por endarterite aórtica e formação de aneurisma micótico.

A 11 years-old male child, with supravalvar aortic stenosis who had as complications aortic endarteritis and mycotic aneurysm formation in the ascending aorta. The aneurysms were diagnosed by a control echocardiogram at the end of an apparently successful clinical treatment of endocarditis. Angiography confirmed the diagnosis and the aneurysms were surgically resected. Six months after surgery the patient is doing well.

[Myocardial revascularization without extracorporeal circulation. Surgical results in 1090 patients]. / Revascularização miocárdica sem circulação extracorpórea. Resultados cirúrgicos em 1090 pacientes.

PURPOSE: To report our experience of 12 years of myocardial revascularization without cardiopulmonary bypass (CPB) and to discuss its expanding surgical indications. METHODS: This technique was employed in 1090 patients, between September/81 and April/93, corresponding to 17.4% of all revascularized patients. Regarding gender, male patients were the majority (73.7%) and ages ranged from 28 to 86 year-old (mean 57). Chronic coronary insufficiency was the main indication (69.4%) and 18.9% of our global experience were operated on during acute ischemia. The average number of grafts per patient was 1.6 and internal mammary artery was utilized in 60.1% of patients. Left anterior descending coronary artery and right coronary artery were the coronary arteries most often treated. RESULTS: Our hospital mortality rate was 2.5% (28/1090) although it was 7% for patients over 70 years. The most common cause of death was cardiogenic shock after failed angioplasty. Postoperative complications included AMI in 4.8%, arrhythmias in 5.5% and pulmonary in 3.2%. Mean of any type of blood derivate utilization was 0.28 units/patients. CONCLUSION: Myocardial revascularization without CPB is a valid alternative in a selected cohort of patients and it is performed with low morbidity and mortality. Nowadays it represents an excellent tactical option for high-risk patients and in failed angioplasties.

Rapid isolation of monosialogangliosides from bovine brain gangliosides by selective-overload chromatography.

A procedure for rapid isolation of monosialogangliosides from purified bovine brain gangliosides has been developed. It utilizes the selective difference in association between monosialogangliosides and polysialogangliosides for the ion-exchange resin Q-Sepharose. When the ion-exchange column is overloaded with a bovine brain ganglioside mixture in the proper ganglioside to column bed-volume ratio, the polysialogangliosides are selectively retained by the column while the monosialogangliosides emerge with the void volume without the use of salt for elution. With the critical ganglioside to bed-volume ratio (1 g:8.32 ml), and an appropriate column bed-height to column radius ratio of 6.9, monosialogangliosides are reproducibly obtained in high purity with greater than 90% yield. The method has been used at both the analytical and preparative scale. We call this separation technique selective-overload chromatography.

[Systemic-pulmonary shunts in congenital heart diseases with decreased pulmonary blood flow: critical analysis of the surgical techniques and immediate results]. / Derivação sistêmico-pulmonar em cardiopatias congênitas com hipofluxo pulmonar: análise crítica da técnica cirúrgica e resultados imediatos.

PURPOSE: To evaluate immediate postoperative results in children with congenital heart disease and decreased pulmonary blood flow who underwent a systemic to pulmonary shunt. PATIENTS AND METHODS: Sixty-four patients underwent surgery, 46.8% (30) of them males with ages from 1 day to 17 years old. They were divided in three groups: I--13 pts (20.3%) who underwent classical Blalock-Taussig (BT) shunt; II--46 pts (71.8%) who underwent modified BT shunts, 34 of them with polytetrafluoroetylene (PTFE) and 12 of them with umbilical vein shunts; III--5 pts (7.8%) with central anastomosis that were made with three different types of graft; 3 PTFE, umbilical vein and bovine mammary artery in one each. The simultaneous procedures were: section and suture of PDA--2, closure of systemic-pulmonary collaterals--3, pulmonary valvotomy--3, right ventricular outflow patch--3, pulmonary branch stenosis (enlargement)--2. RESULTS: In group I there were 4 (30.7%) closures, with two immediate reoperation and a total mortality of 30.7%. In group II there were 4 closures (8.6%) and two reoperations with a mortality of 15.2% (7 cases). In group III there was one graft closure and an overall mortality of 80% (4 pts). When analysing deaths due to the shunt itself the mortality rate was respectively 15.3%, 8.6% and 40.0%. CONCLUSION: Modified BT operation was performed most frequently in our service; it was associated with less closure and mortality than the other types of shunt. It is considered our first alternative for a systemic-pulmonary shunt.