Hepatocellular carcinoma: surgical indications and results

Hepatocellular carcinoma (HCC) is a tumour of increasing incidence that usually arises in cirrhotic liver. Untreated, the prognosis is grim and the only curative treatment is surgical resection. The practical application of segmental surgery to the liver together with the use of ultrasound and other imaging techniques, patient selection criteria and improvements in perioperative technique and postoperative care have contributed to better results in hepatic surgery. Today, less than 10% mortality for resection of cirrhotic livers, with up to 50% 5-year survival rates are to be expected. However, the limits of resection for cure: intrahepatic recurrence makes stringent follow-up necessary. In this way the available modalities of treatment can be applied so as to improve survival. Herein, a current 'state-of-the-art' of surgical indications and results for HCC is given.     

Congenital heart disease: Current indications for antithrombotic therapy in pediatric patients

This report reviews the current status of antithrombotic therapy, including anti-platelet therapy, in pediatric patients with congenital heart disease. The current medications utilized and dose recommendations are emphasized, and indications for their use are reviewed.     

Congenital heart block: clinical features and therapeutic approaches

Isolated congenital heart block is strongly associated with anti-Ro antibodies. It occurs in 2% of anti-Ro antibody positive pregnancies with a recurrence rate of 17-19%. Mortality is high in the first year of life (12-41%) and is predominantly due to dilated cardiomyopathy. A prolonged QTc occurs in 15-22% of cases and minor structural defects such as atrial septal defects and patent arterial ducts are well recognized. The 'mechanical' PR interval can now be measured in utero allowing for the detection of first-degree heart block. Both first and second-degree heart block detected in utero respond to therapy with fluorinated steroids. Complete congenital heart block is not reversible. Progression from a normal PR interval to complete heart block can occur within a week. IVIG is under investigation for the prevention of recurrence of congenital heart block, while dexamethasone should not be used for this purpose due to unacceptable toxicity. Data on the use of fluorinated steroids for established complete heart block is conflicting, although their use in cases where there is evidence of hydrops, poor ventricular function or both is not controversial.     

Cardiac resynchronization for heart failure: background, methods, indications and results

Heart failure is one of the most prevalent diseases in industrialized countries. Although the prognosis of patients with heart failure is still poor, in recent decades new therapies have been investigated in order to improve quality of life and survival. However, up to 30% of the patients with advanced heart failure present disturbances in intraventricular conduction, and this produces asynchrony of ventricular contractility, leading to further deterioration in heart function. Cardiac resynchronization therapy can improve the synchrony of ventricular contractility. Numerous studies have demonstrated the benefits of biventricular stimulation therapy for improving hemodynamic parameters, quality of life, 6-minute walking test performance and functional class in patients with heart failure, ventricular systolic dysfunction and disturbances in intraventricular conduction. Some studies have demonstrated longer survival times in patients treated with cardiac resynchronization plus a defibrillator. Nonetheless, many questions about the benefits of heart resynchronization therapy, site of stimulation and best type of device (pacemaker or defibrillator) remain unresolved.     

Myocarcial revascularisation without extracorporeal circulation: current indications, surgical technique and results

BACKGROUND: This study was undertaken to assess our experience of the evolution, over time, of beating heart surgery in the Cardiologic Center Foundation Monzino. METHODS: From March 1995 to June 2000, 506 patients underwent myocardial revascularization on beating heart: 313 until May 1999, and 193 between June 1999 and June 2000, after the advent of coronary artery stabilizers and shunts, to keep the surgical field bloodless, with minimal motion and continuous myocardial perfusion. Surgical accesss was via a median sternotomy for 408 cases and via a left anterior thoracotomy for 98 cases. RESULTS: The indications by choice increased, from I to II period, from 61% to 83% with special situations in which patients had three-vessel coronary artery disease raised from 33% to 50%, concerning also bypass grafts performed on circumflex artery and right coronary increased. Postoperative mortality in hospital decreased from 1.3% to 0.5% and perioperative IMA (acute myocardial infarction) from 3.8% to 0.5% in patients undertaken to median thoracotomy. Hospital stay decreased from 8 to 7 days about [no significant differences with patients who underwent CPB (cardiopulmonary bypass)]; in patients who underwent to MTS (left anterior minithoracotomy) there was no deaths, IMA decreased from 3.9% to 0% and hospital stay from 6 to 5 days. Grafts patency increased from 92.3% to 100%. CONCLUSIONS: To perform completed revascularisations is possible now even on the beating heart, and also to make precise anastomosis as on pump CABG, in a reproducible and easy way. The beating heart procedure, that is also more economical, might be expanded to all patients, not only high risk patients.     

Liver hemangioma revisited: current surgical indications, technical aspects, results.

BACKGROUND/AIMS: New developments regarding the diagnosis of liver hemangiomas, surgical indication and therapeutic options emerged recently. We reviewed our experience from the point of view of these modern developments. METHODOLOGY: Fifty-seven patients with liver hemangiomas were retrospectively studied. The relationship between size, symptoms and treatment was assessed. RESULTS: Indication for surgery was the size of the lesion in 5 patients, symptomatology in 48 and uncertain diagnosis in 4. Increase in size was noted in four patients. Six patients had associated intraabdominal benign or malignant pathology. The treatment of choice was enucleation. Postoperative complications were noted in 6 patients and mortality was nil. In 3 patients the hemangiomas recurred and were reresected in 2. CONCLUSIONS: Hemangiomas should be resected when larger than 10 cm and when they become symptomatic. With the modern diagnostic work-up, uncertainty of diagnosis as an indication for surgery should be rare. Surgery remains the main treatment, with a low morbidity and mortality if performed in a specialized hepatobiliary unit. Enucleation is the surgical option of choice. In selected cases laparoscopic enucleation can be performed with good results.     

Congenital heart disease,heterotaxia and laterality

Congenital heart disease occurs in about 0,8% of all newborns. Many cardiac malformations occur among relatives and have a polymorphic presentation. The origin of most congenital heart disease is thought to be multifactorial, implying both anomalous expression of genes and the influence of epigenetic factors. However, in a small number of cases, the origin of congenital heart disease has been directly related to chromosomal anomalies or to defects in a single gene. Curiously, defects in a single gene can explain a polymorphic presentation if the anomalous gene controls a basic embryonic process that affects different organs in time and space. Some of these genes appear to control the establishment of laterality.The establishment of the left-right asymmetry starts at the Hensen node. Here, the initial embryonic symmetry is broken by cascades of gene activation that confer specific properties on the left and right sides of the embryo. Although there are variations between species, some basic patterns of gene expression (Nodal, Pitx2) appear to be maintained along the phylogenetic scale. Anomalous expression of these genes induces the heterotaxia syndrome, which usually courses with congenital heart disease. The development of heart malformations is illustrated with the mouse mutant iv/iv, which is a model for the heterotaxia syndrome and the associated congenital heart disease.     

Conduction disturbances and operative results after closure of ventricular septal defects by three different surgical approaches

A total of 135 patients with ventricular septal defect (VSD) under 15 years of age, without associated cardiac lesions, underwent surgical repair by 3 different approaches of trans-pulmonary arteriotomy (PA), right atriotomy (RA) and right ventriculotomy (RA) between 1977 and 1981. Six hospital deaths (4.4%) occurred among the 135 patients. Of 41 children with PA, 53 with RA and 41 with RV, one child (2.4%), one child (1.9%) and 4 children (9.9%) died, respectively. In the trans-PA approach, 8 of 40 children (20.0%) developed complete right bundle branch block (CRBBB), but there was no occurrence of left axis deviation (LAD), transient complete heart block (CHB) and arrhythmias postoperatively. In the RA approach, 11 of 52 children (21.2%) developed CRBBB and 2 (3.8%) had a combination of LAD and CRBBB. Four of 52 children (7.7%) had a combination of LAD, CRBBB and CHB, but CHB has been treated successfully with medication. Three patients (5.8%) developed arrhythmias, such as junctional rhythm. In the trans-RV approach, 11 of 37 children (29.7%) developed CRBBB and 4 of the 37 (10.8%) had the combination of LAD and CRBBB. Two patients (5.4%) had arrhythmias which disappeared one month after surgery. All incidences of conduction disturbances and arrhythmias after closure of VSD were significantly less in the trans-PA than in the trans-RA or in the trans-RV (p less than 0.01). However, in the present study, the development of conduction disturbances and postoperative arrhythmias after closure of VSD was not significantly different between the trans-RA and the trans-RV approach.     

Congenital heart disease

Dr. Frederick K. Heath: Congenital heart disease includes a wide variety of anatomic defects, about one-half of which produce significant physiologic abnormalities. This half, representing about 1 per cent of all organic heart disease and about 2 per cent of that seen in children, recently has become the center of great physiologic and surgical interest. For advances in vascular surgery have made it possible to achieve dramatic and often life-saving results in this group of patients. Coincidently, investigation by means of the intravenous catheter had progressed so far that precise diagnostic and postoperative follow-up information could be obtained. This was important since it soon became obvious that clinical appraisal based only on physical findings and statistics gave neither accurate diagnosis nor quantitative information regarding cardiac function. Important, too, is the use of radiographic technics whereby the abnormality may be visualized.The four cases presented serve to illustrate present day technics and their limitations, how data obtained by catheterization technics are interpreted, and representative results of surgical therapy. They also emphasize the frequent occurrence of multiple defects.By means of cardiac catheterization with simultaneous measurement of the blood oxygen content and pressures in the various chambers it is possible by application of the Fick principle to calculate cardiac output, to define the systemic and pulmonary circulation in terms of volume flow per unit of time, and to estimate, when present, the direction of shunt flow. In connection with pressure measurements it is to be remembered that an increase in pressure may result from obstruction to flow, e.g., stenosis of the pulmonary artery, in which case the rate of flow will be decreased; or on the other hand, it may simply be due to an increased rate of flow per se. It is clear that information both as to pressure and rate of flow are important for interpretation.Accurate anatomic and physiologic estimations are of invaluable aid in deciding whether or not operative interference should be helpful. Thus in simple patent ductus arteriosus an excellent result is to be expected. Of twenty-six uncomplicated cases of this condition operated upon by Dr. Humphreys only one death occurred postoperatively and only one proven recurrence was noted. When the patent ductus arteriosus is complicated by other defects, the outlook depends upon the nature of these complications. In this group the operative mortality and results are decidedly less favorable.When indicated, i.e., cyanotic heart disease, the creation of an artificial ductus, as carried out by Dr. Blalock, may relieve cyanosis but it also increases the pulmonary blood flow and the work of the heart. Whether eventual cardiac hypertrophy and failure, pulmonary congestion and polycythemia may be postoperative sequelae is not yet clear. These findings were observed in one patient thought to have a tetrad of Fallot plus a patent ductus arteriosus from birth; yet in patients receiving an artificial ductus the disappearance of polycythemia has been encountered.     

Congenital heart disease and heart failure

The syndrome of heart failure in adult non-congenital heart disease patients includes myocardial disease and ventricular dysfunction. In the presence of congenital abnormalities the cause of heart failure is often multi-factorial and can be a result of the underlying anomaly, surgical intervention, or ventricular dysfunction. Despite the possible clinical similarities, the two conditions are fundamentally different. In congenital heart disease the neurohormonal system is already abnormal even in the absence of clinical manifestations of heart failure and, in many cases, exercise intolerance is related to cyanosis. The approach to heart failure management in the two etiologies might be similar. Preventative attempts to preserve ventricular function in coronary or valve disease parallels early reparative therapy in congenital heart disease Pharmacological therapy is common for the two conditions, despite the limited number of evidence-based recommendations for congenital diseases. In drug-resistant patients, cardiac electrical resynchronization is an established therapy for treating ventricular asynchrony in non-congenital heart failure sufferers, but has only recently been adopted in selected congenital cases. Due to this, congenital heart disease patients are managed in highly specialized unites in close cooperation with cardiologists and surgeons. The ideal follow-up protocol for such patients remains to be determined, particularly in those individuals with subclinical signs of residual cardiac dysfunction. Heart Fail Monit 2008;6(1):2-8.     

Congenital heart disease in Down's syndrome patients: a decade of surgical experience

Patients with Down's syndrome represent a significant subset of patients with congenital heart disease. Fifty-five patients with Down's syndrome have undergone surgical treatment for congenital heart disease at our institution in the past decade. Twenty-six had atrioventricular canal, 11 had ventricular septal defect, 7 had secundum atrial septal defect, 7 had tetralogy of Fallot, 3 had primum atrial septal defect and 1 patient had double outlet right ventricle. The thirty day mortality following operative intervention was 16.4%. Mortality was highest for tetralogy of Fallot followed by atrioventricular canal and ventricular septal defect. Long term mortality for all lesions was 27.3% over our follow-up period which averaged 33 months. Thirty day mortality compared similarly to previous reports of surgically treated Down's syndrome patients. When compared to our patients without Down's syndrome, the Down's population did not exhibit an increased risk for surgical treatment of congenital heart disease.     

Congenital heart disease: Cor biloculare

The interesting features of this case, aside from its great rarity, are the presence of the four auriculoventricular valves which represent the primitive endocardial cushions, the hyperplastic eustachian valve, the common pulmonary vein and the unusual venous circulation of the lung. An examination of the roentgenogram of the chest revealed only a single large artery going upward and descending to the right.     

Congenital heart disease and pregnancy

In Europe and North America, a dramatic fall in the incidence of rheumatic fever and rheumatic heart disease has coincided with advances in medical and surgical management of congenital heart disease and has resulted in a shift in the relative incidence of these two categories of cardiac disorders in women of child-bearing age. This review deals with pregnancy and congenital heart disease—unoperated and operated. Central to this topic is the intricate interplay between maternal circulatory and respiratory physiology and maternal congenital heart disease, and the effects of this interplay upon the fetus which is exposed to risks that threaten its intrauterine viability and to risks that subsequently express themselves as developmental defects or transmitted congenital malformations of the heart or circulation.