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Biomedical subjects

F J Puga

Publications and source records attributed to F J Puga.

At least 55 records · Page 3Linked to original sources

Therapeutic implications of intraoperative pressure measurements after the Fontan operation.

Central venous pressure (CVP) and left atrial pressure (LAP) were monitored continuously for the first 72 hours postoperatively in 32 patients who underwent a Fontan operation in whom preoperative measurements of the pulmonary artery index were available. Integrated mean values were generated for each patient for the following time frames: (1) the first 12 hours after operation, (2) the first 24 hours after operation, (3) postoperative day 2, and (4) postoperative day 3. We found no difference in the CVP, LAP, or transpulmonary gradient, derived as CVP-LAP, measured in the operating room at the completion of the operation versus that measured on the third postoperative day: CVP, 18 +/- 2 mmHg versus 19 +/- 3 mmHg; LAP, 10 +/- 2 mmHg versus 10 +/- 3 mmHg; and transpulmonary gradient, 8 +/- 2 mmHg versus 8 +/- 2 mmHg. The combined incidence of hospital mortality and postoperative takedown associated with the Fontan repair was 12.5%. These findings suggest that a poor hemodynamic result from the Fontan operation can be predicted from intraoperative pressure measurements, because the CVP, LAP, and transpulmonary gradient are unlikely to change significantly in the early postoperative period. Therefore, a decision to take down or fenestrate the repair can reasonably be made in the operating room or the early postoperative period.

Adolescent↗

Imaging strategies for vascular rings.

Thirty-nine patients have undergone operation for relief of tracheoesophageal compression resulting from vascular rings and related entities at the Mayo Clinic. Nineteen patients had a double aortic arch, 11 patients had a right aortic arch with an aberrant left subclavian artery, 5 patients had a left aortic arch with an aberrant right subclavian artery, 2 patients had a pulmonary artery sling, 1 patient had a right aortic arch with mirror-image branching and a left ligamentum arteriosum, and 1 patient had a left aortic arch, a right descending aorta, and a right ductus arteriosus. Diagnostic examinations included chest radiography, barium esophagography, angiography, and, more recently, transthoracic echocardiography, computed tomography, and magnetic resonance imaging. A comparison among the various diagnostic techniques used in 12 patients during the last 12 years showed that angiography (n = 7), magnetic resonance imaging (n = 5), and computed tomography (n = 3) were the most reliable, as they always accurately delineated the anatomy. However, in the 6 patients who underwent transthoracic echocardiography, 1 of whom was an older child and 2 of whom were adults, the vascular abnormality was described correctly only once; in the other 5 patients, the results were false-negative or the technique failed to visualize the relevant vascular structures sufficiently. Currently, magnetic resonance imaging is our imaging technique of choice for the delineation of the vascular and tracheal anatomy in patients suspected of having a vascular ring.

Adolescent↗

Aortic intramural course of the left coronary artery in dextro-transposition of the great arteries.

Three patients with dextro-transposition of the great arteries and an aortic intramural left coronary artery are described. A review of our patients with dextro-transposition of the great arteries and those reported in the literature suggests that an aortic intramural course of the left coronary artery or left anterior descending coronary artery must be assumed when the artery arises from the right (posterior) sinus of Valsalva, distal to the right sinotubular junction, or at the commissure between the right and left (anterior) sinuses and courses between the great arteries.

Aorta, Thoracic↗

Enteric protein loss after the Fontan operation.

OBJECTIVE: The current study was undertaken to assess the frequency of excessive enteric protein loss and protein-losing enteropathy in the relatively early period after the Fontan operation. DESIGN: Protein excretion was determined in 26 of 27 consecutive patients who underwent the Fontan procedure between January and June 1990 at the Mayo Clinic. MATERIAL AND METHODS: At two testing intervals during the first 4 months after the Fontan operation, alpha 1-antitrypsin clearance and fecal alpha 1-antitrypsin concentration studies were done. RESULTS: All results were normal for the first postoperative test period (2 to 8 weeks). For the second study period, all 17 patients tested had normal alpha 1-antitrypsin clearances. One of the 17 patients had an appreciably increased fecal alpha 1-antitrypsin concentration and transient protein-losing enteropathy. CONCLUSION: Excessive enteric protein loss and protein-losing enteropathy are relatively uncommon during the first 4 months after the Fontan operation.

Adolescent↗

Preoperative diagnosis of coronary artery anatomy in dextrotransposition of the great arteries.

BACKGROUND: Preoperative diagnosis of the coronary artery pattern in dextrotransposition of the great arteries is important because deviation from the usual pattern may influence the surgical strategy. METHODS: For assessment of the value of angiography and echocardiography in this setting, we analyzed the preoperative echocardiographic and angiographic findings in 74 patients with dextrotransposition of the great arteries in whom an arterial switch operation was performed. RESULTS: Two-dimensional echocardiography established a diagnosis of the coronary artery pattern in 40 of the 74 patients; that diagnosis was correct in 38-30 of 32 (94%) with the usual coronary artery pattern and 8 (100%) with an atypical pattern. Angiography established a diagnosis of the coronary artery pattern in 52 patients; that diagnosis was correct in 50-41 of 42 (98%) with the usual coronary artery pattern and 9 of 10 (90%) with an atypical pattern. The accuracy of echocardiography in diagnosing the coronary artery pattern in dextrotransposition of the great arteries in this series was 95% and that of angiography was 96%. CONCLUSION: Both echocardiography and angiography can establish the diagnosis of the coronary artery anatomy in most patients. Echocardiography is routinely performed in infants with dextrotransposition of the great arteries and is proving to be increasingly sensitive for diagnosing the coronary artery anatomy; the role of angiography may evolve to be supplementary, especially in cases in which the coronary artery anatomy is not clearly demonstrated by echocardiography.

Child, Preschool↗

Supravalvular aortic stenosis. Long-term results of surgical treatment.

To determine long-term outcome after operation for supravalvular aortic stenosis, we reviewed the case histories of 80 patients who had repair of the localized form (group A) (n = 67) or diffuse form (group B) (n = 13) from 1956 to 1992, including 31 patients with the Williams-Beuren syndrome. Ages ranged from 7 months to 54 years (mean = 12.6 years). Forty-six patients had one or more associated cardiovascular anomalies; the most common was aortic valve stenosis (33.8%). Eighteen patients had 22 previous cardiovascular operations, and 28 patients had one or more additional anomalies repaired during their initial procedure at our institution. In group A, the aortic root was enlarged with a teardrop-shaped patch (n = 61) or a pantaloon-shaped patch (n = 6). In group B, patch enlargement of the aorta was confined to the root (n = 4) or extended into the ascending aorta or aortic arch (n = 7); one patient had a graft placed between the ascending and descending thoracic aorta and one patient had a left ventricular-aortic conduit. There were no deaths in group A; two patients in group B in whom patch enlargement was confined to the aortic root died during the operation (2.5%). Follow-up extended to 33.4 years (mean = 14.2 years); there were five late deaths in group A and one in group B. Survival excluding operative mortality was 94% at 10 years and 91% at 20 years. All patients were in functional class I or II. There was no significant difference between patients with a teardrop-shaped or pantaloon-shaped patch in terms of late gradient, survival, or aortic insufficiency. By Cox multivariate model, the only independent predictor of late death for all patients was associated aortic valve disease (p = 0.02), which was also a risk factor for late reoperation (p = 0.02). In group B, overall survival was better in patients who received an extended patch versus aortic root patch only (p = 0.02). We reached the following conclusions: (1) Associated aortic valve disease was strongly correlated with late death and need for reoperation. (2) Both the teardrop-shaped and pantaloon-shaped patch techniques provide excellent long-term relief of localized supravalvular gradients and preservation of aortic valve competence. (3) In diffuse supravalvular aortic stenosis, aortic enlargement should be extended into the ascending aorta or beyond as required to relieve the gradient; some patients may require a graft or conduit.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

Pulmonary artery size and clinical outcome after the modified Fontan operation.

The maximum cross-sectional area of the central pulmonary arteries indexed to the body surface area (pulmonary artery index [PAI]) was measured preoperatively from angiograms in 173 patients evaluated for a Fontan-type operation between 1981 and June 1989. Of these, 34 patients underwent another palliative procedure, 8 primarily on the basis of small pulmonary arteries (PAI, 106 to 167 mm2/m2). The mean PAI of this group was significantly smaller than the mean PAI of the remaining 139 patients who underwent a Fontan operation (136 +/- 20 versus 310 +/- 113 mm2/m2) (p < 0.001). The patients who underwent a Fontan operation were evaluated according to three overlapping end points: (1) hospital death or takedown of repair (12.2%), (2) early failure (cumulative death or takedown of repair within 6 months of operation) (16.5%), and (3) early failure or persistent effusions (33.8%). With regard to these end points, no significant difference in pulmonary artery size could be found between patients having a favorable or unfavorable outcome. However, among a low-risk subset of 30 patients with tricuspid atresia, those with "early failure or persistent effusions" had significantly smaller pulmonary arteries than those with a good outcome (PAI, 185 +/- 47 versus 276 +/- 83 mm2/m2) (p < 0.01). The postoperative transpulmonary gradient of the 8 patients with the smallest pulmonary arteries who underwent a Fontan operation (all PAIs < 170 mm2/m2) was significantly greater than that of the rest of the study group (9.88 +/- 2.3 versus 8.13 +/- 2.3 mm Hg) (p < 0.04).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Mediastinal paraganglioma: a surgical experience.

Between October 1960 and January 1991, 5 male and 9 female patients (mean age, 44 +/- 19 years) were seen at the Mayo Clinic because of mediastinal paraganglioma. Nine patients presented with hypertension. In these and an additional patient, the tumors were biochemically active. In 13 patients, the mediastinal mass was localized preoperatively. All patients with functioning tumors received alpha-adrenergic and beta-adrenergic blockade preoperatively. Total resection was performed in 11 patients and partial resection in 2. In 1 patient, the tumor was found at postmortem examination. Five lesions were grossly invasive and nine were noninvasive. Blood pressure returned to normal in 6 of the 8 hypertensive patients whose tumors were totally removed. In 4, catecholamine excretion normalized as well. Tumor multicentricity occurred in 6 patients. In 7 patients, the disease took a malignant course (metastases in 5 and progression of unresectable lesions in 2). The probability of 10-year survival after resection was 50% in the group with malignant tumors, and there were no deaths in the group with benign lesions. Analysis of the DNA cellular content showed no correlation between the ploidy pattern and the behavior of the disease.

Adolescent↗

Cardiorespiratory response to exercise after the Fontan operation: a serial study.

OBJECTIVES: The purpose of this study was to assess whether there is deterioration of aerobic capacity over time after the Fontan operation in individual patients. BACKGROUND: We previously observed that maximal aerobic capacity after the Fontan operation was lower in older patients than in younger patients. It was unclear whether this represented a decrease in aerobic capacity with time after operation or was a function of studying patients of different ages at different times postoperatively. METHODS: All patients who had more than one postoperative exercise study were included. There were 25 patients (19 male, 6 female), aged 3.8 to 39 years at the time of the operation. The first exercise test was performed, on average, 2.2 years after the Fontan operation, and the last exercise test was performed, on average, 5.9 years (range 1.8 to 13) after the operation. In 11 patients, coronary sinus drainage was left on the pulmonary venous side. Five patients had had a previous Glenn operation. Exercise was performed to exhaustion with use of a 3-min incremental cycle protocol. RESULTS: Exercise duration, oxygen uptake, blood pressure, respiratory rate, minute ventilation, pulmonary blood flow index, exercise factor, ST-T wave changes and the prevalence of arrhythmias were similar during the first and last tests. Exercise systemic arterial blood oxygen saturation decreased from the first to the last postoperative test (p < 0.006) regardless of age. The percent of predicted heart rate, at rest and during maximal exercise, decreased more in older patients from the first to the last test (p < 0.05 for rest and exercise). CONCLUSIONS: In this select group of patients, exercise tolerance remained relatively unchanged over the range of 13 years after the Fontan operation. Heart rate at rest and during maximal exercise decreased more than predicted for age in older patients. A small but significant progressive decrease in systemic arterial blood oxygen saturation was found. The former might represent abnormal sinus node function in patients with functional single ventricle, and the latter might represent a tendency toward development of abnormal ventilation/perfusion patterns resulting from the development of small pulmonary arteriovenous fistulas or an effect of position of the coronary sinus postoperatively.

Adolescent↗

Aortopulmonary window: factors associated with early and late success after surgical treatment.

Between 1953 and 1990, 19 patients, who were from 7 weeks to 27 years old, underwent surgical correction of aortopulmonary window at the Mayo Clinic. Associated cardiac anomalies were present in nine patients (47%). At operation, extracorporeal circulation was used in all except one patient. In seven patients, division and primary closure were done. In four patients, the defect was closed by direct suture through a transpulmonary approach. In eight patients, the defect was closed with a patch through a transpulmonary or transaortic approach. Four deaths (21%) occurred intraoperatively or immediately postoperatively. All four patients had undergone division of the aortopulmonary window before 1962, and three of them had a pulmonary vascular resistance (Rp) that was 7.3 U.m2 or more and a ratio of Rp to systemic vascular resistance (Rp/Rs) that exceeded 0.6. One patient with an Rp of 11.8 U.m2 and an Rp/Rs of 0.72 died 16 years postoperatively. Statistical analysis of risk factors indicated that early year of operation (P = 0.022), division of the aortopulmonary window versus transaortic or transpulmonary closure (P = 0.009), and a high Rp/Rs (P = 0.021) were significantly associated with perioperative death. All patients with a preoperative Rp of 8 U.m2 or less, an Rp/Rs of less than 0.4, and no major associated cardiac anomalies were in functional class I (New York Heart Association) postoperatively. Our study confirms that infants with aortopulmonary window should undergo operation early, before irreversible pulmonary vascular changes have developed. Patients with an Rp/Rs that exceeds 0.4 should be thoroughly assessed to determine their operability.

Adolescent↗

Cor triatriatum: diagnosis, operative approach, and late results.

From May 1960 to January 1992, 13 patients with cor triatriatum underwent surgical correction at the Mayo Clinic. Their ages ranged from 7 months to 57 years. Four patients had isolated cor triatriatum; three others had an associated patent foramen ovale or atrial septal defect. Six patients had major associated cardiac anomalies. In patients who had isolated cor triatriatum or associated anomalies with high pulmonary blood flow, the mean gradient across the left atrial diaphragm was 24.4 mm Hg, in comparison with 11.6 mm Hg in patients who had cor triatriatum and a connection between the common pulmonary venous chamber and the right atrium or associated cardiac anomalies that restricted pulmonary blood flow. In all 11 patients who underwent angiography, echocardiography, or both at our institution, the correct diagnosis was established preoperatively. Currently, echocardiography is the procedure of choice for diagnosing cor triatriatum. The diaphragm was excised through a left atrial approach in seven patients and through a right atriotomy in six. All associated anomalies were corrected. One critically ill patient who underwent an emergency operation in 1963 died early postoperatively, and one patient with chromosomal abnormalities and multiple cardiac defects died 2 months after an uneventful postoperative course. At the time of follow-up of the 11 long-term survivors, 9 were in New York Heart Association functional class I, and 2 (both of whom had major associated cardiac anomalies) were in class II. Postoperative angiography or echocardiography (in nine patients) showed no residual interatrial shunt or recurrent left atrial diaphragm.

Adolescent↗

Surgical treatment of vascular rings: the Mayo Clinic experience.

From 1947 through 1992, 37 Mayo Clinic patients underwent operation for the relief of tracheoesophageal obstruction that resulted from vascular rings and related entities. Of the 37 patients, 18 had a double aortic arch, 11 had a right aortic arch with an aberrant left subclavian artery, 4 had a left aortic arch with an aberrant right subclavian artery, 2 had a pulmonary artery sling, 1 had a right aortic arch with mirror-image branching and a left ligamentum arteriosum, and 1 had a left aortic arch, a right descending aorta, and a right ductus arteriosus. Symptoms consisted of stridor, recurrent respiratory infections, and dysphagia. The anomaly was approached through a left thoracotomy in 31 patients, through a right thoracotomy in 4, and through a median sternotomy in 2. Only one early postoperative death (3%) and no late deaths occurred. At long-term follow-up (maximal duration, 45 years), three patients had residual symptomatic tracheomalacia, one of whom required right middle and lower lobectomy for recurrent pneumonia. Magnetic resonance imaging is the imaging technique of choice for accurate delineation of the vascular and tracheal anatomy. When patients are symptomatic, vascular ring should be repaired. The surgical risk is minimal, and the long-term results are excellent.

Adolescent↗

Modified Fontan operation in the presence of anomalies of systemic and pulmonary venous connection.

BACKGROUND: The purpose of this report is to outline technical maneuvers dictated by anomalies of systemic and/or pulmonary venous connection in the performance of the Fontan procedure. METHODS AND RESULTS: Between 1975 and 1990, 104 patients (60 male, 44 female) with anomalies of systemic and/or pulmonary venous connection underwent a modified Fontan procedure at the Mayo Clinic. Mean age was 9.7 +/- 5.7 years. Isolated anomalies of the systemic venous connection were identified in 46 patients, isolated anomalous pulmonary venous connections in 4, and a combination of the two in 54. Previous palliative operations had been performed in 93 patients. Surgical repair was accomplished by atrial septation or placement of an intra-atrial conduit combined with cavopulmonary anastomosis if required. Survival by Kaplan-Meier, including operative mortality, was 55.7% at 10.3 years, not significantly different from the overall survival of the Fontan population. By the proportional hazards general linear model procedure, insufficiency of the systemic atrioventricular valve, preoperative mean pulmonary pressure greater than 15 mm Hg, and pulmonary artery resistance index greater than 4 U.m2 were associated with higher mortality. Five patients required reoperation for pulmonary venous obstruction (1 patient), revision of the atrial baffle (1 patient), revision of the intra-atrial conduit (2 patients), and replacement of the systemic atrioventricular valve (1 patient). CONCLUSIONS: We conclude that the modified Fontan operation can be successfully performed in this subset of patients, with long-term results comparable to those obtained in patients with normal systemic and pulmonary venous connection.

Cardiac Surgical Procedures↗

Surgical treatment of discrete and tunnel subaortic stenosis. Late survival and risk of reoperation.

BACKGROUND: Although membranectomy, with or without septal myotomy or myectomy, has been the accepted method for treatment of fixed subaortic stenosis, controversies remain regarding operative methods and uncertainties regarding recurrence of subaortic obstruction and development of aortic insufficiency after repair. METHODS AND RESULTS: To determine late survival and risk of reoperation, we reviewed 169 patients who, between 1957 and 1992, had repair of discrete (n = 108) and tunnel (n = 61) subaortic stenosis. One hundred fifty-four patients had their primary operation for subaortic stenosis at the Mayo Clinic, and 15 patients had their primary operation elsewhere. Among patients having initial repair at our institution, membranectomy was performed in 52 patients (33.8%), membranectomy plus myotomy in 24 patients (15.6%), membranectomy plus myectomy in 71 patients (46.1%), myectomy plus a Konno-Rastan procedure in 5 patients (3.2%), and myectomy plus a modified Konno-Rastan procedure in 2 patients (1.3%). For all patients, early mortality was 4.7% (2.7% since 1961), and during follow-up extending to 29 years, there have been 16 late deaths. Twenty-six patients underwent a second or third operation for recurrent left ventricular outflow obstruction, including 11 of the 154 who had their primary operation at the Mayo Clinic (7.1%). Among the 21 patients who had a discrete lesion at initial repair and required reoperation, 19 (92%) were noted to have tunnel obstruction at reoperation. At late follow-up, the left ventricular outflow tract gradient was higher in patients with tunnel versus discrete obstruction (33 +/- 5 versus 24 +/- 17 mm Hg, P < .04), and 10-year survival was poorer (79% versus 91%, P < .02). Ten-year survival was worse in patients with tunnel lesions and associated cardiac anomalies versus those with isolated tunnel subaortic stenosis (64% versus 92%, P < .005). Some degree of aortic valve insufficiency was seen at late follow-up in 26% of patients, but in most cases this was mild. For patients with discrete subaortic stenosis, risk of late aortic insufficiency was 38.6% after isolated membranectomy, 27.8% after membranectomy and myotomy, and only 7.3% after membranectomy and myectomy (P < .004). Progression of aortic insufficiency requiring aortic valve replacement occurred in only 6 patients. CONCLUSIONS: Our results support the use of myectomy in conjunction with membranectomy for discrete subaortic stenosis. For restenosis and tunnel obstruction, more complete relief of subaortic stenosis by extended resection or a modified or classical Konno-Rastan procedure may improve late survival and reduce the incidence of recurrent subaortic stenosis and late aortic valve insufficiency.

Adolescent↗

Pulmonary microthrombi. Caveat for successful modified Fontan operation.

We reviewed medical records from eight patients (4 to 29 years of age) with a functional single ventricle and pulmonary microthrombosis as observed on open lung biopsy specimens. Hemodynamic assessment before biopsy revealed pulmonary hypertension (mean pulmonary artery pressure 19 to 53 mm Hg) in the seven patients tested and severely increased pulmonary arteriolar resistance (6 to 13 U.m2) in three of the five patients in whom these measurements were performed. Pulmonary blood flow varied (< 4 L/min per square meter in two patients and > 5 L/min per square meter in four patients), and the pulmonary/systemic blood flow ratio was low or normal (0.6 to 1.1) in five of six cases. Hemoglobin values were increased (16.4 to 22 gm/dl) in seven of eight patients. Lung biopsy specimens revealed thrombotic obstructive lesions in 56% (range 28% to 96%) of muscular pulmonary arteries. Coexistent medial hypertrophy was absent or mild in all but one specimen, and none had features of plexogenic pulmonary arteriopathy. Microscopic pulmonary thrombi accounted for unexpected pulmonary hypertension, precluding the Fontan operation, in five patients and unsuccessful Fontan operation in two. Clinically significant thrombi should be considered in patients with severe polycythemia (hemoglobin value > 20 gm/dl) and unobtainable or unreliable pulmonary blood flow or pulmonary resistance determinations.

Adolescent↗