Cryoablation: a novel approach to neurolysis of the ganglion impar

BACKGROUND: The main goal in the surgical repair of total anomalous pulmonary venous drainage is to reestablish a wide patent connection between the common pulmonary vein and the left atrium. Several techniques have been proposed for achieving this objective, each of which has advantages and disadvantages. The superior approach between the superior vena cava and the ascending aorta was introduced in 1976 for the repair of supracardiac forms of total anomalous pulmonary venous drainage, but it often provides a less than optimum exposure, particularly in tiny infants. We proposed a modification of this approach that includes division of the ascending aorta and offers excellent exposure. METHODS: Seventeen patients (15 neonates and 2 infants) with supracardiac total anomalous pulmonary venous drainage (n = 13) or mixed forms of total anomalous pulmonary venous drainage (n = 4) underwent surgical repair with the use of the modified superior approach. Circulatory arrest was not required in 10 patients and the mean cross-clamp time was 32.5 +/- 13.8 minutes. RESULTS: There was 1 postoperative death resulting from intractable pulmonary hypertension in a compromised infant who was referred to our unit receiving extracorporeal membrane oxygenation. One patient with common hypoplasia underwent reoperation twice at 2 months and then 3 months after the first procedure. All the other patients had a smooth postoperative course, and midterm evaluation showed a widely patent anastomosis between the common vein and the left atrium. CONCLUSIONS: The modified superior approach for the repair of supracardiac total anomalous pulmonary venous drainage can be useful to enhance exposure during surgical repair and may contribute to improved patient outcome.     

The surgical importance of coronary sinus orifice atresia

Coronary sinus orifice atresia is rare. We describe two cases, one with an atrioventricular septal defect and another with supracardiac totally anomalous pulmonary venous drainage. The association with the latter has not been described previously. The importance of diagnosing the defect is emphasized and surgical treatment is discussed.     

In vitro metabolism of a new 4-hydroxycoumarin anticoagulant. Structure of an unusual metabolite

A case of common atrium with anomalous systemic and pulmonary venous connections and pulmonary stenosis is described. Surgical correction was performed by pulmonary valvotomy and the insertion of an intra-atrial pericardial baffle. To our knowledge this is the first reported case of surgical correction of total anomalous connections of both systemic and pulmonary veins. The embryologic development of this condition is discussed.     

Myocardial recovery through ECMO after repair of total anomalous pulmonary venous connection: the importance of left heart unloading

A 7-day-old boy who had been placed on extracorporeal membrane oxygenation on his second day of life developed biventricular failure after undergoing surgical repair of a supracardiac variant of total anomalous pulmonary venous connection. Extracorporeal membrane oxygenation was again necessary for postoperative cardiopulmonary support. However, severe left ventricular failure made it imperative to leave the vertical vein open during support in order to decrease pressure on the left ventricle. The patient was successfully weaned from extracorporeal membrane oxygenation on day 8 after surgery and discharged from the hospital on day 23.     

Treatment of local recurrence of rectal cancer

We report on 2 infants with Ullrich-Turner syndrome in whom partial anomalous pulmonary venous drainage was diagnosed noninvasively by color-coded Doppler sonography. Several patients with the combination of anomalous drainage of one or more pulmonary veins and Ullrich-Turner syndrome have been described in the literature. However, in the majority of those previously reported cases the diagnosis of partial anomalous pulmonary venous drainage was established by angiography during cardiac catheterization performed for confirmation of other cardiovascular malformations. Our patients show that partial anomalous pulmonary venous drainage can be diagnosed easily in neonates and young infants, as long as this anomaly is taken into consideration.     

Is vertical vein ligation necessary in repair of total anomalous pulmonary venous connection?

BACKGROUND: In the repair of total anomalous venous connection, vertical vein ligation is recommended to eliminate left-to-right shunting. However, the small left heart chambers may not always tolerate the immediate increase in blood flow after combined repair and vein ligation. METHODS: A retrospective review of 23 infants and children undergoing correction of total anomalous pulmonary venous connection was undertaken to determine whether vertical vein ligation is a necessary component of successful surgical repair. In 14 patients this vein was ligated, whereas in 9 it was left patent. Six patients who underwent ligation and 5 who did not had pulmonary venous obstruction before operation. RESULTS: The operative mortality rate was 36% (5 of 14 patients) for the ligated group compared with 0% (0 of 9 patients) for the nonligated group (p = 0.06). All deaths occurred in patients with preoperative obstruction and a low mean left atrial pressure, and four of the deaths were directly attributable to left heart failure. Follow-up echocardiography in patients in whom the vertical vein was not ligated revealed adequate cardiac function and no residual left-to-right flow through the previously patent venous conduit. CONCLUSION: Vertical vein ligation during the repair of total anomalous pulmonary venous connection is not routinely necessary and actually may be undesirable in patients with preoperative obstruction, in whom the left heart chambers are particularly small.     

A transesophageal Doppler echo-color study of pulmonary venous flow before and after the correction of valvular defects

AIM OF THE STUDY: To verify changes of pulmonary venous flow pattern before and after surgical or percutaneous correction of valvular heart disease. METHODS: The pulmonary venous flow pattern was studied by transesophageal echocardiography in 27 patients affected with heart valve disease (11 mitral insufficiency, 10 mitral stenosis, 2 aortic stenosis and 4 pulmonary stenosis), before and after surgical or percutaneous correction. Pulmonary venous flow velocity variables measured included peak systolic and diastolic flow velocities (VmaxS and VmaxD), systolic and diastolic velocity time integrals (IS and ID) and their respective ratios (VmaxS/VmaxD and IS/ID). Paired Student's t-test was used for analysis of data; a p value < 0.05 was considered statistically significant. RESULTS: In mitral stenosis and insufficiency, as well as in pulmonary stenosis, the VmaxS/VmaxD and IS/ID ratios were constantly < 1. Aortic stenosis, on the contrary, showed a normal preoperative pattern of pulmonary venous flow, which did not change after correction. All other successful corrections (17 surgeries, 4 angioplasties) were characterised by an increase of VmaxS/VmaxD and IS/ID ratios. (Mitral stenosis: VmaxS/VmaxD 0.80 +/- 0.31 vs 1.4 +/- 0.5, p = 0.006; IS/ID 0.86 +/- 0.77 vs 1.62 +/- 0.62, p = 0.016. Severe mitral insufficiency: VmaxS/VmaxD -0.71 +/- 0.32 vs 1.19 +/- 0.32, p < 0.0001; IS/ID 0.41 +/- 0.19 vs 1.04 +/- 0.31, p = 0.006. Moderate mitral insufficiency: VmaxS/Vmax D 0.38 +/- 0.04 vs 0.95 +/- 0.06, p = 0.001; IS/ID 0.32 +/- 0.05 vs 0.95 +/- 0.07, p = 0.02. Pulmonary stenosis: VmaxS/VmaxD 0.43 +/- 0.23 vs 1.09 +/- 0.35, n.s. e IS/ID 0.49 +/- 0.34 vs 0.92 +/- 0.65, n.s.). Failure to return to a normal pulmonary venous pattern was observed in the 2 cases of partially successful mitral valvuloplasty (one of which was subsequently transformed into a mitral valve replacement with immediate normalisation of the pattern) and in the 2 cases of incomplete relief of a pulmonary stenosis after pulmonary valvuloplasty. CONCLUSIONS: Though preliminary, these observations suggest a high sensitivity of this method and, therefore, a possible role of pulmonary venous pattern studies in the assessment of the efficacy of treatment in mitral and pulmonary valve disease.     

Three unusual complications resulting from attempted repair of partial anomalous pulmonary venous drainage

The correction of shunts resulting from partial anomalous pulmonary venous drainage has become an accepted surgical procedure. Surgical complications, other than those that were purely postoperative, have been rare. The present report details the case histories of three patients with unusual complications resulting from this type of surgery. Unilateral pulmonary venous obstruction and repeated infections occurred in one patient. In another, obstruction of the superior vena cava resulted. In the third patient, an indaequate operation was performed when the site of partial anomalous pulmonary venous drainage into the coronary sinus was not recognized initially at the time of surgery.     

The kleeblattsch?del anomaly in Apert syndrome: intracranial anatomy, surgical correction, and subsequent cranial vault development

We present a case of Apert syndrome in which intracranial anomalies of the cranial base were localized to the lesser wings of the sphenoid and sphenoid ridge. The lesser wings of the sphenoid were displaced superiorly to follow the fused coronal sutures bilaterally, where they met at a single point on the skull vertex. Careful preoperative study of the intracranial anatomy in the kleeblattsch?del anomaly led to a surgical plan for early correction of the anomaly. The present report indicates that an aggressive approach to the correction of the kleeblattsch?del anomaly beginning early in infancy can result in normalization of the trilobar skull configuration. Although this approach can correct the kleeblattsch?del anomaly, 3.5-year follow-up in this patient with Apert syndrome demonstrates progressive turricephaly despite repeated cranial vault remodeling. Although the trilobar skull configuration can be corrected through early surgical intervention, the long-term correction of progressive turricephaly in patients with Apert syndrome remains an unsolved problem.     

Total anomalous pulmonary venous return complicating portoenterostomy for biliary atresia

Cardiovascular anomalies such as absent inferior vena cava and preduodenal portal vein are reported in cases of biliary atresia and make hepatic portoenterostomy a technical challenge. The authors present the case of a severe cardiac anomaly that significantly altered the functional outcome of a Kasai procedure. Baby M., an 8-week-old boy born with total anomalous pulmonary venous return (TAPVR), underwent hepatic portoenterostomy for biliary atresia. Over the next 3 months he remained icteric and febrile, and failed to gain weight. After multiple antibiotic treatments for suspected cholangitis, he underwent reexploration of the portoenterostomy, with no improvement in his overall condition. His prognosis was considered dismal because correction of the cardiac anomaly is associated with a high mortality rate (> 90%). The cardiac surgeon agreed to attempt a cure of the TAPVR, provided liver transplantation is contemplated if the patient survived. Within 48 hours postoperatively, his hepatic function had improved drastically. He became afebrile, had an improved appetite and weight gain, and was finally discharged 203 days after admission. One year later, he is thriving and remains anicteric. The exact reason for this drastic improvement is not well understood, but the right-sided cardiac failure caused by the TAPVR had a significant effect on the functional outcome of the portoenterostomy.     

Provoked enuresis-like episodes in healthy children 7 to 12 years old

A 15-year-old boy with tetralogy of Fallot and pulmonary atresia without central pulmonary arteries who was successfully treated using a staged approach is presented. The first stage consisted of the creation of central pulmonary arteries. In the second stage, the continuity between the right ventricle and the pulmonary arteries was established with closure of the ventricular septal defect. Some patients previously considered to be unsuitable candidates for correction may be successfully repaired using this approach.     

Popliteal venous aneurysm: report of two cases and review of the world literature

Two new cases of popliteal venous aneurysm are reported and added to the 22 other cases of popliteal venous aneurysm available for review. Both patients were first seen with acute pulmonary embolism and were treated with thrombolytic therapy followed by anticoagulation. Each had recurrent venous thromboembolism before discovery of the popliteal venous aneurysm. One popliteal venous aneurysm was diagnosed with phlebography and the second with venous duplex imaging, confirmed with phlebography. Both were surgically corrected with tangential aneurysmectomy and lateral venorrhaphy. Twenty-four cases of popliteal venous aneurysm are now available for review. Seventy-one percent (17 of 24) presented with pulmonary embolism, 88% (21 of 24) were saccular, and 96% (23 of 24) were located in the proximal popliteal vein. All but two were diagnosed by ascending phlebography. Three patients received no treatment: in two of these the outcome was not documented and the third had occasional pain. Two patients received anticoagulation without subsequent operative repair and both died of recurrent pulmonary emboli. Operative correction resulted in a 75% patency rate with 21% complications, most of which were related to postoperative anticoagulation. No patient who was operated on had subsequent pulmonary embolism, and there were no operative deaths. We suggest that all patients who have pulmonary embolism have lower-extremity venous duplex imaging. All popliteal venous aneurysms should be surgically repaired, inasmuch as nonoperative therapy results in recurrent thromboembolism and an unacceptably high mortality rate. Tangential aneurysmectomy with lateral venorrhaphy is the recommended procedure.     

Total anomalous pulmonary venous connection. Results of a small consecutive series

The surgical experience in 13 infants with total anomalous pulmonary venous connection (TAPVC) between 1987 and 1991 is reviewed. The age vary from 2 days to 35 months with a weight at intervention from 2.130 kg to 5.400 kg. The types of TAPVC were supracardiac in 4 patients, cardiac in 4, and infracardiac in 5. Seven patients (54%) were operated on in emergency. Cardiopulmonary bypass consisted of profound hypothermia and total circulatory arrest in 8 patients (60%) and continuous hypothermic bypass with low flow for the remaining 5 patients (40%). There was no operative death. The follow-up ranges from 21 to 58 months, mean 40 months. There was one reoperation. All the patients were asymptomatic and the height growth percentile is less than 5% in 20%, and the weight growth percentile is less than 5% in 30%. The early repair of infants born with TAPVC can be done with low morbidity with a good prospect on medium term follow-up.     

Surgical treatment of solitary or common heart ventricle

On the basis of 24 cases reported in the literature and 2 personal observations of the authors the indications for surgical correction of a single ventricle of the heart are discussed. Two different surgical techniques are described: 1) haemodynamic correction of the defect comprising a closure of the venous atrioventricular valve, oversewing the proximal pulmonary artery, and anastomosing the right atrium and the distal pulmonary artery; 2) radical correction--creation of the ventricular septum. The variants of the disease permitting a radical correction are indicated, the results of surgery, the causes of complications and mortality are discussed.     

Total anomalous pulmonary venous connection in neonates and young infants: repair in the current era

Total anomalous pulmonary venous connection has been one of the more challenging congenital heart defects in newborns and young infants despite improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care. Since 1981, 30 patients with total anomalous pulmonary venous connection have undergone primary total correction. Mean age at operation was 28 +/- 6 days and mean weight, 3.3 +/- 0.7 kg. Essential features of the surgical approach in these small patients included early surgical intervention, profound hypothermia with total circulatory arrest, cardioplegic myocardial preservation, and a wide posterior anastomosis. Operative mortality was 13% +/- 6%. All four deaths were in patients having emergency operation within 24 hours of the surgical consult and requiring ventilator support preoperatively. The mean follow-up is 47 +/- 7 months. There have been two late deaths, and the 7-year survival rate is 79% +/- 8%. There have been two reoperations, and 91% +/- 6% of the patients are reoperation free at 7 years. Only 1 of the 24 surviving patients is symptomatic. Growth in survivors is closely monitored. The height growth percentile is less than 5% in 15% +/- 8% of survivors and the weight growth percentile, less than 5% in 17 +/- 8%. During the past decade, with a consistent surgical approach to neonates and infants with total anomalous pulmonary venous connection, it has been possible to achieve low early mortality, low attrition, and excellent late results.     

Auditory evoked neuromagnetic response in cerebrovascular diseases: a preliminary study

OBJECTIVE: Postoperative deep venous thrombosis and pulmonary embolus are major causes of morbidity and mortality in patients undergoing surgical procedures. In contrast to other surgical fields, the incidence of these life-threatening conditions has not been studied in our specialty. The purposes of this study were to elucidate the incidence of deep venous thrombosis and pulmonary embolus in patients after otolaryngologic operations and to identify specific risk factors that may contribute to the development of these conditions. METHODS: A retrospective analysis was done of 12,805 total operations on adults done by the Department of Otolaryngology at our institution from January 1987 to December 1994 to determine the number of patients in whom postoperative deep venous thrombosis and pulmonary embolus developed. Patients in whom a postoperative thromboembolic event developed after an otolaryngologic surgical procedure were identified by the medical records department with use of an abstracting database. This search cross-referenced disease-specific codes for otolaryngologic procedures with the codes for deep venous thrombosis and pulmonary embolus to identify the 34 patients in this report. Results (rounded to the nearest decimal point) were then categorized according to the different subspecialties within otolaryngology, and appropriate statistical analysis tests were performed on the resulting data. RESULTS: Thirty-four patients with postoperative deep vein thrombosis were identified during the study period, for an overall incidence of 0.3%. Of these 34 patients, 24 also had a pulmonary embolus for an overall incidence of 0.2%. The incidence of deep venous thrombosis (and pulmonary embolus) in the subspecialties was as follows: head and neck surgery, 0.6% (0.4%); otology/neurotology, 0.3% (0.2%); head and neck trauma and plastic surgery, 0.1% (0.1%); and general otolaryngology, 0.1% (0.04%). Only the patient's age and the presence or absence of pneumatic compression devices were identified as independent risk factors for the development of a thromboembolic event. CONCLUSIONS: Postoperative pulmonary embolus is a rare occurrence in the field of otolaryngology-head and neck surgery. When it does occur, it causes significant morbidity and increases the cost of care for that patient. We discuss our approach to categorizing patients into low-, intermediate-, and high-risk groups, as well as prophylaxis against pulmonary embolus.     

Duration of pulmonary venous atrial reversal flow velocity and mitral inflow a wave: new measure of severity of cardiac amyloidosis

Transmitral Doppler flow patterns of patients with cardiac amyloidosis evolve from an early impaired relaxation to an advanced restrictive pattern. This reflects increasing severity of diastolic dysfunction and hence left ventricular filling pressures. The duration of the pulmonary venous atrial reversal flow was recently shown to exceed that of the mitral inflow A wave in patients with left ventricular end-diastolic pressure greater than 15 mm Hg. The objective of this study was to assess the utility of this index as a measure of the severity of cardiac amyloidosis. Comprehensive transthoracic 2-dimensional and pulsed-wave Doppler echocardiograms of the pulmonary venous and transmitral flows were made of 23 patients (10 women) with biopsy-proven diagnosis of primary systemic amyloidosis and of 49 subjects as age-matched normal controls. The amyloidosis group was divided into non-restrictive and restrictive subgroups on the basis of the patients' transmitral inflow deceleration time (>150 and < or =150 ms, respectively). The durations of the pulmonary venous atrial reversal and mitral inflow A wave were measured, and the differences between the flow durations were compared with the control and published data in the nonrestrictive and restrictive groups. The mean duration of the pulmonary venous atrial reversal was significantly longer in the amyloid than the control group (P < .01). The mean duration of the mitral inflow A wave was significantly shorter in the restrictive group than both the nonrestrictive and the control groups (P < .05). The duration of the pulmonary venous atrial reversal exceeded that of the mitral inflow A wave in all patients with cardiac amyloidosis. The difference in duration between pulmonary venous atrial reversal and mitral inflow A wave was significantly greater in the amyloidosis group compared with the normal group, and this index varied significantly within the amyloid group between the abnormal relaxation and the restrictive groups. The difference in the duration between the pulmonary venous atrial reversal and the mitral inflow A wave is a reliable index of diastolic function and can be used to assess the severity of cardiac amyloidosis.     

Pulmonary regurgitation in large atrial shunts without pulmonary hypertension

Seven patients with pulmonary regurgitation (PR), normal pulmonary artery (PA) pressures and large left-to-right atrial shunts are reported. Six had secundum atrial septal defects (ASD) and one had anomalous pulmonary venous drainage. These comprised 4% of 180 patients with atrial shunts and normal PA pressures. Pulmonary regurgitation was diagnosed clinically by mid-frequency diastolic decrescendo murmurs beginning after the pulmonic component of the second heart sound, and diagnoses were confirmed by catheterization. In two patients who had serial preoperative catheterizations over 8 and 16 years, PR progressed in one and was present only on the second study in the other. All patients underwent shunt correction, at which time the pulmonic anulus and artery appeared dilated, but the pulmonic valves were normal and did not require revision. In all patients the PR murmur disappeared after shunt correction alone, and on chest X-ray both PA and overall heart size decreased. Although it is known that pulmonary regurgitation occurs with atrial septal defects and pulmonary hypertension, the present study demonstrates that it also occurs with high flow atrial shunts, in which setting it has different implications and is reversible with shunt correction alone.     

The clamshell approach for the surgical treatment of complex cardiopulmonary pathology in infants and children

BACKGROUND: The surgical approach to children with complex cardiovascular and pulmonary anomalies is still controversial. Staged operations through multiple incisions are often performed in this setting. OBJECTIVE: The different applications and clinical advantages of a bilateral thoracosternotomy approach to complex cardiothoracic disease requiring surgical repair were reviewed retrospectively. METHODS: Between January 1993 and June 1995, 33 patients, aged between 2 months and 17 years (mean 7.8 +/- 5.3) underwent surgical treatment of complex cardiovascular or pulmonary disease using a clamshell approach. Twenty-one patients (64%) had undergone 1-5 previous surgical procedures (mean 2.5 +/- 1.0/patient). The technique involved supine position placement, submammary incision, access to the pleural space bilaterally through the fourth intercostal space and transverse division of the sternal body. RESULTS: Four groups of patients were operated on via this approach: (1) patients undergoing lobar, lung or heart-lung transplantation (40%); (2) patients undergoing repair of tetralogy of Fallot/pulmonary atresia (36%); (3) patients with previously corrected miscellaneous procedures (12%), including completion of Fontan, one-stage repair of left main bronchial stenosis and atrial septal defect, one-stage repair of partial anomalous pulmonary venous connection and aortic coarctation, and repair of congenital pulmonary venous stenosis. There were two early (< 30 days) deaths, giving a perioperative mortality of 6% for the entire series. Complications included postoperative hemorrhage in 4 patients (12%), prolonged ventilation time due to mechanical failure in 4 (12%). There were no wound infections. Analysis of complications by group showed the lung transplant group to be more affected (18% of patients experienced complications). Except for 2 infants undergoing complete unifocalization and presently awaiting completion of repair of tetralogy of Fallot/pulmonary atresia, in the remaining 31 (94%) a definitive surgical treatment could be performed in one-stage. CONCLUSIONS: The bilateral thoracosternotomy allows optimal exposure of all intrathoracic anatomic structures making one-stage surgical repair possible in a variety of complex cardiovascular and pulmonary anomalies. Early mortality and technique-related morbidity do not differ from those reported with the conventional approaches to the different disease conditions. A wider application of the clamshell approach for the management of complex intrathoracic pathology in infants and children is advocated.     

Estimation of left ventricular filling pressures using two-dimensional and Doppler echocardiography in adult patients with cardiac disease. Additional value of analyzing left atrial size, left atrial ejection fraction and the difference in duration of pulmonary venous and mitral flow velocity at atrial contraction

OBJECTIVES: The purpose of this study was to determine whether left atrial size and ejection fraction are related to left ventricular filling pressures in patients with coronary artery disease. BACKGROUND: In patients with coronary artery disease, left ventricular filling pressures can be estimated by using Doppler mitral and pulmonary venous flow velocity variables. However, because these flow velocities are age dependent, additional variables that indicate elevated left ventricular filling pressures are needed to increase diagnostic accuracy. METHODS: Echocardiographic left atrial and Doppler mitral and pulmonary venous flow velocity variables were correlated with left ventricular filling pressures in 70 patients undergoing cardiac catheterization. RESULTS: Left atrial size and volumes were larger and left atrial ejection fractions were lower in patients with elevated left ventricular filling pressures. Mean pulmonary wedge pressure was related to mitral E/A wave velocity ratio (r = 0.72), left atrial minimal volume (r = 0.70), left atrial ejection fraction (r = -0.66) and atrial filling fraction (r = -0.66). Left ventricular end-diastolic and A wave pressures were related to the difference in pulmonary venous and mitral A wave duration (both r = 0.77). By stepwise multilinear regression analysis, the ratio of mitral E to A wave velocity was the most important determinant of pulmonary wedge (r = 0.63) and left ventricular pre-A wave (r = 0.75) pressures, whereas the difference in pulmonary venous and mitral A wave duration was the most important variable for both left ventricular A wave (r = 0.75) and left ventricular end-diastolic (r = 0.80) pressures. The sensitivity of a left atrial minimal volume > 40 cm3 for identifying a mean pulmonary wedge pressure > 12 mm Hg was 82%, with a specificity of 98%. CONCLUSIONS: Left atrial size, left atrial ejection fraction and the difference between mitral and pulmonary venous flow duration at atrial contraction are independent determinants of left ventricular filling pressures in patients with coronary artery disease. The additive value of left atrial size and Doppler variables in estimating filling pressures and the possibility that left atrial size may be less age dependent than other mitral and pulmonary venous flow velocity variables merit further investigation.