Biventricular repair in neonates with hypoplastic left heart complex

PURPOSE: We previously developed an inpatient regimen that consisted of infusional fluorouracil (5-FU), epirubicin, and cisplatin (ECisF), with a response rate of 86% in advanced breast cancer. The current phase II 2:1 randomized study investigated whether cyclophosphamide can be substituted for cisplatin (ECycloF) to reduce toxicity and allow the regimen to be administered on an outpatient basis without loss of efficacy. PATIENTS AND METHODS: Ninety-six women (median age, 49 years; range, 28 to 73) with breast cancer (59 metastatic, 37 locally advanced) received continuous infusional 5-FU (200 mg/m2/d via Hickman line) and six cycles of epirubicin (60 mg/m2 every 21 days) with either cyclophosphamide 600 mg/m2 every 21 days (38 metastatic, 24 locally advanced) or cisplatin 60 mg/m2 every 21 days (21 metastatic, 13 locally advanced). There were no significant differences in patient characteristics between these groups. RESULTS: ECycloF was better tolerated than ECisF in terms of lethargy (P = .005), stomatitis (P = .008), plantar palmar erythema (P = .02), constipation (P < .001), thrombosis (P = .0014), and nausea and vomiting (P = .05). Although there was a trend toward more anemia and leukopenia with ECisF (P =. 1), there was no significant difference in the rates of infection. Efficacy was comparable in terms of overall response (69% v 68%), complete response (CR; 13% v 15%), and median progression-free survival (9 v 8 months). CONCLUSION: ECycloF is an outpatient regimen with a lower incidence of severe nonhematologic toxicity than inpatient ECisF; it has comparable efficacy and is considerably more economical.     

Performance of the lift ventricle in left ventricular obstructive cardiomyopathy

Whether volume expansion influences NaC1 reabsorption by the diluting segment of the nephron remains a matter of controversy. In the present studies this question has been examined in normal unanesthetized dogs, undergoing maximal water diuresis. Free water clearance (CH2O/GFR) has been used as the index of NaC1 reabsorption in the diluting segment. Three expressions have been employed for "distal delivery" of NaC1: a) V/GFR, designated as the "volume term"; b) (CNa/GFR + CH2O/GFR), the "sodium term;" and c) (CC1/GFR + CH2O/GFR), the "chloride term". The validity of these terms is discussed. Three techniques were used to increase distal delivery: 1) the administration of acetazolamide to dogs in which extracellular fluid (ECF) volume was not expanded (grop 1); 2) "moderate" volume expansion (group 2); and 3) "marked" volume expansion (group 3). CH2O/GFR increased progressively with rising values for "distal delivery" regardless of which term was used to calculate the latter. With all three delivery terms, differences in distal NaC1 reabsorption emerged between the two volume-expanded groups, though only with the "chloride" term did substantial differences also emerge between the nonexpanded group 1 dogs and both volume-expanded groups. In group 1, values for CH2O/GFR increased in close to a linear fashion up to distal delivery values equal to 24% of the volume of glomerular filtrate. However, at high rates of distal delivery the rate of rise of CH2O/GFR was less in group 2 than in group 1 and the depression of values was even greater in group 3. Within the limits of the techniques used, the data suggest that volume expansion inhibits fractional NaC1 reabsorption in the diluting segment of the nephron in a dose-related fashion. The "chloride" term was found to be superior to the "volume" and "sodium" terms in revealing these changes.     

Monitoring of cardiac function in patients with a left ventricle assist device

In complex therapy of acute or chronic cardiac failure, the use of valve-free centrifugal pumps or artificial heart ventricles takes place. In these cases, simultaneous monitoring of cardiac and pump performance is very important. In this study, an ultrasound dilution method based on the registration of two dilution curves after intravenous injection of isotonic and hypertonic sodium solution was developed. Comparison of total cardiac output (TCO), which included pump and heart output measured by thermodilution and ultrasound (TCOus) dilution, yielded TCOus = 1.11TCOth - 0.54 (n = 13).     

Hamartomatous malformation of the left ventricle associated with sudden death

We describe unusual left ventricular cardiac lesions in a 17 year old boy who died suddenly during exertion. These consisted of two grossly evident regions of deficient myocardium, containing cavernous spaces which represented exaggerated intertrabecular regions of the left ventricular cavity. Dense fibro-elastotic tissue was deposited around these spaces along with a variable admixture of mature adipose tissue, fibrous tissue and blood vessels. The etiology of these presumably congenital developmental abnormalities is obscure. The lesions most probably represent a hamartomatous malformation, which is a poorly documented pathological entity.     

Pseudoaneurysm of the left ventricle following repair for ventricular septal perforation

The patient was a 64-year-old man who was treated surgically for an infarct-related ventricular septal perforation. Pseudoaneurysm of the left ventricle was recognized on the 38th postoperative day. Emergency surgery was performed. It seemed that insufficient resection of the infarcted myocardium was performed during the initial surgery to avoid narrowing the ventricular dimension by direct closure of the left ventricle, but this resulted in pseudoaneurysm of the left ventricle. Left ventricular free wall plasty with a patch should be performed during the initial surgery.     

Technique of transplantation for hypoplastic left heart syndrome with left superior vena cava

The presence of a left superior vena cava in infants with hypoplastic left heart syndrome complicates the technical performance of orthotopic cardiac transplantation. In this report we describe a modification of the recipient cardiectomy to maintain patency of the left superior vena cava, leaving the recipient coronary sinus as a conduit for it into the right atrium and avoiding complex venous reconstructions.     

Obstetric management of 219 infants with hypoplastic left heart syndrome

OBJECTIVE: The objective of this study was to describe the obstetric parameters from 219 deliveries of infants with hypoplastic left heart syndrome. STUDY DESIGN: The Pediatric Cardiovascular Surgery Database at the University of Michigan was searched, and cases of neonates with the diagnosis of hypoplastic left heart syndrome were found. Obstetric records were then reviewed. RESULTS: One hundred sixty-one infants (74%) were delivered vaginally and 58 (26%) were delivered by the cesarean route. The mean gestational age at delivery was 38.9 weeks. Mean Apgar scores at 1 and 5 minutes were 7 and 8, respectively. Ninety percent were delivered at term and 10% were delivered before 37 weeks. The diagnosis of hypoplastic left heart syndrome was made antenatally in 82 cases (37%) and neonatally in 137 cases (63%). In the antenatal group the mean gestational age at diagnosis was 27 weeks. Karyotype analysis was performed in 32 of all cases (15%), with 8 fetuses revealing an abnormal karyotype. Seven cases were 45,X and 1 was trisomy 21. CONCLUSION: Staged reconstruction surgery has markedly improved survival for neonates born with isolated hypoplastic left heart syndrome. Our review suggests that, aside from determining the karyotype, no further obstetric interventions seem warranted. While awaiting spontaneous labor at term, the planned mode of delivery should be vaginal, with cesarean delivery performed for routine obstetric indications only.     

A successful repair of infective endocarditis accompanied by a pseudoaneurysm of the left ventricle

The present paper reports a successful surgical treatment of a 47-year-old male with a pseudoaneurysm of the left ventricle. The patient has also been administered Penicillin G for 5 months to treat endocarditis. Cardiac catheterization showed severe aortic stenosis and a pseudoaneurysm of the left ventricle which was dilating in systole. The patient underwent patch closure of the pseudoaneurysm whose ostium was situated at the miral-aortic inter valvular fibrosa followed by aortic valve replacement and direct closure of a right Valsalva sinus aneurysm. His postoperative course was uneventful. The patient had no recurrence of endocarditis nor malfunction of the prosthetic valve for one year postsurgery. This is the first report in Japan of successful surgical treatment of a pseudoaneurysm of the left ventricle due to perforation of the miral-aortic intervalvular fibrosa after endocarditis.     

Appearance of the interventricular septum in obstructive lesions of the left ventricular outflow tract

Measurements of the thickness of the septum were made in 26 cardiac specimens and in 13 patients. There were 10 normal specimens, five with aortic stenosis, two with asymmetric septal hypertrophy, four with hypertensive cardiovascular disease, and five with idiopathic cardiomyopathy. Patient measurements were obtained by visualizing the septum during simultaneous left and right ventriculography on angiograms obtained in the left anterior oblique projection. Four patients were normal, five had aortic stenosis, and four had a symmetric septal hypertrophy. Measurements derived from normal cardiac specimens and angiographic appearance suggested that the normal septum is a smooth-walled structure with right and left ventricular surfaces parallel, diverging only slightly at the apex of the ventricle. In aortic stenosis and idiopathic cardiomyopathy, the septum tends to be biconvex with maximal thickening in its middle third. Hypertensive cardiovascular disease produces uniform septal thickening, resembling an exaggeration of normal. However, in asymmetric septal hypertrophy no consistent patterns of hypertrophy or septal thickening are evident; bulging can be present at any point along the left ventricular surface of the septum.     

Abnormal left ventricular filling after neonatal repair of congenital heart disease: association with increased mortality and morbidity

BACKGROUND: The presence of mid-diastolic flow reversal on the mitral valve Doppler inflow indicates abnormal left ventricular filling. To determine whether mid-diastolic flow reversal predicts outcome in patients undergoing repair or palliation of neonatal congenital heart disease, we reviewed the echocardiograms and medical records of 40 patients with either left ventricular outflow obstruction or transposition of the great arteries. METHODS: All patients underwent surgical repair; transposition of the great arteries (TGA) = 17, coarctation of the aorta (CoA) = 14, interrupted aortic arch (IAA) = 8, and aortic stenosis (AS) = 1. The presence of mid-diastolic flow reversal was determined by pulsed Doppler interrogation of the mitral valve on preoperative and postoperative echocardiograms. RESULTS: Preoperative echocardiograms showed diastolic flow reversal in only 5 patients; 1 of 1 with AS and 4 of 14 with CoA. Twenty-one of 40 patients showed postoperative diastolic flow reversal; 1 of 1 with AS, 8 of 8 with IAA, 1 of 14 with CoA, and 11 of 17 with TGA. Postoperative mid-diastolic flow reversal 1 to 3 days after surgery was associated with higher mortality rate: 7 of 21 patients with diastolic flow reversal and 0 of 19 without diastolic flow reversal died. Patients with diastolic flow reversal who survived had longer intensive care unit (26.2 +/- 13.5 days vs 7.1 +/- 4.1 days, P <.001) and hospital (57.4 +/- 38.8 days vs 14.8 +/- 5.2 days, P <.05) stays. CONCLUSION: Mid-diastolic flow reversal is an indicator of prolonged hospital stay and mortality in patients with left ventricular outflow tract obstruction or TGA.     

Tracking and motion analysis of the left ventricle with deformable superquadrics

We present a new approach to analyse the deformation of the left ventricle of the heart based on a parametric model that gives a compact representation of a set of points in a 3-D image. We present a strategy for tracking surfaces in a sequence of 3-D cardiac images. Following tracking, we then infer quantitative parameters which characterize: left ventricle motion, volume of left ventricle, ejection fraction, amplitude and twist component of cardiac motion. We explain the computation of these parameters using our model. Experimental results are shown in time sequences of two modalities of medical images, nuclear medicine and X-ray computed tomography (CT). Video sequences presenting these results are on the CD-ROM.     

Successful repair of total anomalous pulmonary venous connection and incomplete endocardial cushion defect associated with left isomerism

Successful repair of a 8-month-old girl with polysplenia was reported. The cardiovascular anomalies were TAPVC (II b), incomplete ECD, interruption of inferior vena cava with hemiazygos continuation, bilateral superior vena cava, and left superior vena cava draining into the coronary sinus. Cardiopulmonary bypass was established with ascending aortic perfusion and caval cannulation. A left superior vena cava was directly cannulated after establishing partial bypass. In this case the left pulmonary vein drained into the right atrium near the orifice of the coronary sinus, so the atrial septal flap was made and sutured between the orifice of the left pulmonary vein and the coronary sinus in order to avoid late pulmonary vein obstruction. Then, atrium was separated by an intraatrial baffle which was sutured to the atrial septal flap. Recently, it becomes possible to surgical repair of polysplenia syndrome according to the advancements of the diagnostic methods, cardiopulmonary bypass, and the technique of the open heart surgery.     

Estimates of regional work in the canine left ventricle

Assessment of the magnitude of regional myocardial work requires knowledge of regional fiber stress and fiber shortening. The theoretical development and experimental validation of a method is presented which used values of estimated active and passive fiber stress according to a fluid-fiber model, and measured fiber strain values. This enables the construction of regional stress-strain diagrams, a regional analog of the pressure-volume area model by Suga and co-investigators, which can be linked to regional oxygen consumption. In the left ventricle, either normally or asynchronously activated, the method yields reliable data on strain and active and passive fiber stress. The relation between estimated regional work and myocardial oxygen demand is in quantitative agreement with previously reported relations between global oxygen demand and measured pressure-volume area. During coronary artery occlusion, however, these values were less reliable, which might be due to inaqdequate knowledge of the (passive) material properties of the myocardium.     

Systolic time intervals of the left ventricle in neuromuscular diseases

In 64 patients having neuromuscular disease and in 11 healthy persons, preejection period (PEP), ejection time index (LVETI) and PEP/LVET ratio of the left ventricle were studied by indirect method. PEP was significantly longer, ejection time index was significantly shorter and PEP/LVET ratio was significantly higher in patients with progressive muscular dystrophy than healthy persons. In 75% of patients having this disease LVETI was shortened and only in 30% of them Ecg abnormalities could be detected. These findings are postulated to be due to diffusely decreased contractility of the left ventricular heart muscle in progressive muscular dystrophy.     

An experience of the modified Norwood's operation for hypoplastic left heart syndrome with aberrant origin of right subclavian artery and persistent left superior vena cava--the procedure without total circulatory arrest and cardiac arrest

We reported a successful case of the modified Norwood operation for a 21-day-old neonate with hypoplastic left heart syndrome (MS and AS) associated with an aberrant right subclavian artery and a persistent left superior vena cava. The modified Norwood operation was performed without total circulatory arrest and Cardiac arrest. A 4 mm Gore-Tex graft, which was anastomosed between the right carotid artery and the right pulmonary artery for systemic-pulmonary shunt, was used for cerebral perfusion during aortic arch reconstruction. Coronary perfusion was performed with a small cannula placed on the relatively large ascending aorta during anastomosis between the main pulmonary artery and the ascending aorta. Equine pericardial patch was used for aortic arch reconstruction and the ascending aorta was directly anastomosed to a part of the main PA. Postoperative course was uneventful and postoperative MRI revealed no stenosis of the aortic arch and the pulmonary artery.