Peripartum anesthetic management in patients with left ventricular noncompaction: a case series and review of the literature.

BACKGROUND: This retrospective review focuses on peripartum anesthetic management and outcome of a series of five pregnant women with left ventricular noncompaction (LVNC). METHODS: The Mayo Clinic Advanced Cohort Explorer medical database was utilized to identify women diagnosed with LVNC who had been admitted for delivery at the Mayo Clinic in Rochester, Minnesota, between January 2001 and September 2021. Echocardiograms were independently reviewed by two board-certified echocardiographers, and those determined by both to meet the Jenni criteria and/or having compatible findings on magnetic resonance imaging (MRI) were included. Electronic medical records were reviewed for information pertaining to cardiac function, labor, delivery, and postpartum management. RESULTS: We identified 44 patients whose medical record included the term "noncompaction" or "hypertrabeculation" and who had delivered at our institution during the study period. Upon detailed review of the medical records, 36 did not meet criteria for LVNC, and three additional patients did not receive the diagnosis until after delivery, leaving five patients with confirmed LVNC who had undergone six deliveries during the study interval. All five patients had a history of arrhythmias or had developed arrhythmias during pregnancy. One patient underwent emergency cesarean delivery due to sustained ventricular tachycardia requiring three intra-operative cardioversions. CONCLUSIONS: This case series adds new evidence to that already available about pregnancies among women with LVNC. Favorable obstetrical outcomes were achievable when multidisciplinary teams were prepared to manage the maternal and fetal consequences of intrapartum cardiac arrhythmias and hemodynamic instability.

Clinical profile and outcome of idiopathic restrictive cardiomyopathy.

BACKGROUND: Idiopathic restrictive cardiomyopathy is a poorly recognized entity of unknown cause characterized by nondilated, nonhypertrophied ventricles with diastolic dysfunction resulting in dilated atria and variable systolic function. METHODS AND RESULTS: Between 1979 and 1996, 94 patients (61% women) 10 to 90 years old (mean, 64 years) met strict morphological echocardiographic criteria for idiopathic restrictive cardiomyopathy, mainly dilated atria with nonhypertrophied, nondilated ventricles. None had known infiltrative disease, hypertension of >5 years' duration, or cardiac or systemic conditions associated with restrictive filling. Nineteen percent were in NYHA class I, 53% in class II, and 28% in class III or IV. Atrial fibrillation was noted in 74% of patients and systolic dysfunction in 16%. Follow-up (mean, 68 months) was complete for 93 patients (99%). At follow-up, 47 patients (50%) had died, 32 (68%) of cardiovascular causes. Four had heart transplantation. The death rate compared with actuarial statistics was significantly higher than expected (P<0.0001). Kaplan-Meier 5-year survival was 64%, compared with expected survival of 85%. Multivariate analysis using proportional hazards showed that the risk of death approximately doubles with male sex (hazard ratio [HR] = 2.1), left atrial dimension >60 mm (HR = 2.3), age >70 years (HR = 2.0), and each increment of NYHA class (HR = 2.0). CONCLUSIONS: Idiopathic restrictive cardiomyopathy or nondilated, nonhypertrophic ventricles with marked biatrial dilatation, as defined morphologically by echocardiography, affects predominantly elderly patients but can occur in any age group. Patients present with systemic and pulmonary venous congestion and atrial fibrillation and have a poor prognosis, particularly men >70 years old with higher NYHA class and left atrial dimension >60 mm.

Noncardiac surgery in Eisenmenger syndrome.

OBJECTIVES: The purpose of this study was to evaluate the morbidity and mortality associated with noncardiac surgery (NCS) in patients (pts) with Eisenmenger syndrome. BACKGROUND: Noncardiac surgery in pts with Eisenmenger syndrome is associated with increased cardiovascular complications. METHODS: Fifty-eight pts with Eisenmenger syndrome (17M, 41F aged 18 to 69 years (mean 41 years) who had been followed for up to 41.5 years (mean 9.3 years) were retrospectively evaluated for any NCS done at > or = 17 years of age. RESULTS: Twenty-four pts had a total of 28 NCSs at an age of 17 to 55 years (mean 29 years) including 9 tubal ligations, 3 neurosurgeries, 3 cholecystectomies, 3 hysterectomies, 3 vasectomies, and 1 each spinal fusion, appendectomy, eye enucleation, hernia repair, hand surgery, tonsillectomy and therapeutic abortion. There were two deaths (7%), one following spinal fusion and the other following appendectomy at another institution. Fourteen of these NCSs were performed at our institution, including 11 under general anesthesia. The duration of anesthesia varied from 75 to 525 min (mean 165 min). All pts remained in sinus rhythm. The lowest systolic blood pressure (BP) ranged from 78 to 125 mm Hg. Of those 11 pts, 9 were extubated immediately after surgery and 2 needed dopamine. Ten patients were discharged without any complications, including 3 within 1 day of surgery. One death occurred 10 days following spinal fusion. This pt had the longest anesthesia (525 min) and an intraoperative systolic BP as low as 78 mm Hg. She also needed the largest fluid administration (6,475 cc) in addition to postoperative mechanical ventilation and dopamine. CONCLUSIONS: Adult pts with Eisenmenger syndrome are at increased risk with NCS, but with current/modern techniques, the risk of death is less than previously thought. In the vast majority of cases, NCS can be undertaken without substantial morbidity, and early extubation is achievable. However, even with relatively minor surgery, significant complications, including death, can occur. Referral to major centers with expertise in the care of pts with Eisenmenger syndrome is advisable.

Coarctation of the aorta: outcome of pregnancy.

OBJECTIVES: The study sought to determine the outcome of pregnancy in women with coarctation of the thoracic aorta. BACKGROUND: Patients with coarctation of the thoracic aorta are expected to reach childbearing age, but data on the outcome of pregnancy in this population are limited. METHODS: The Mayo Clinic database was reviewed for women of childbearing age (> or =16 years old) with a diagnosis of aortic coarctation evaluated from 1980 to 2000. Spectrum of cardiovascular disease, surgical history, and obstetrical and neonatal outcomes were determined. RESULTS: Fifty women with coarctation had pregnancies: 30 had coarctation repair before pregnancy, 10 had repair after pregnancy, 4 had repair both before and after pregnancy, and 6 had no history of repair. The 50 women had 118 pregnancies resulting in 106 births. There were 11 miscarriages (9%), 4 premature deliveries (3%), and 1 early neonatal death; 38 deliveries (36%) were by cesarean section. Of the 109 offspring, 4 (4%) had congenital heart disease. A patient with Turner syndrome died of a Stanford type A dissection at 36 weeks of pregnancy. Nineteen women (38%) were known to have hemodynamically significant coarctation during pregnancy (gradient > or =20 mm Hg). Fifteen women (30%) had hypertension during their pregnancy, 11 of whom (73%) had hemodynamically significant coarctation during that time (8 with native and 3 with residual/recurrent coarctation). CONCLUSIONS: Major cardiovascular complications were infrequent but continue to be a source of concern for patients with coarctation who become pregnant. Systemic hypertension during pregnancy was common and related to the presence of a significant coarctation gradient.

Left atrial appendage thrombus is not uncommon in patients with acute atrial fibrillation and a recent embolic event: a transesophageal echocardiographic study.

OBJECTIVES: The objective of this study was to determine the frequency of left atrial thrombus in patients with acute atrial fibrillation. BACKGROUND: It is commonly assumed but unproved that left atrial thrombus in patients with atrial fibrillation begins to form after the onset of atrial fibrillation and that it requires > or = 3 days to form. Thus, patients with acute atrial fibrillation (i.e., < 3 days) frequently undergo cardioversion without anticoagulation prophylaxis. METHODS: Three hundred seventeen patients (250 men, 67 women; mean [+/- SD] age 64 +/- 12 years) with acute (n = 143) or chronic (n = 174) atrial fibrillation were studied by two-dimensional transesophageal echocardiography. RESULTS: Left atrial appendage thrombus was present in 20 patients (14%) with acute and 47 patients (27%, p < 0.01) with chronic atrial fibrillation. In patients with a recent embolic event, the frequency of left atrial appendage thrombus did not differ between those with acute (5 [21%] of 24) and those with chronic (12 [23%] of 52, p = NS) atrial fibrillation. Patients with acute versus chronic atrial fibrillation, respectively, did not differ (p = NS) in mean age (64 +/- 13 vs. 65 +/- 11 years), frequency of concentric left ventricular hypertrophy (32% vs. 26%), hypertension (32% vs. 41%), coronary artery disease (35% vs. 39%), congestive heart failure (43% vs. 48%), mitral stenosis (4% vs. 7%) or mitral valve replacement (1.4% vs. 6%). The minimally detectable difference in proportions between patients with acute and chronic atrial fibrillation based on a power of 0.80 and base proportion of 0.20 was 14%. CONCLUSIONS: Left atrial thrombus does occur in patients with acute atrial fibrillation < 3 days in duration. The frequency of left atrial thrombus in patients with recent emboli is comparable between those with acute and chronic atrial fibrillation. These data suggest that patients with acute atrial fibrillation for < 3 days require anticoagulation prophylaxis or evaluation by transesophageal echocardiography before cardioversion and should not be assumed to be free of left atrial thrombus.

Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography.

OBJECTIVE: This study sought to demonstrate that with proper technique, identification of the normal and abnormal pulmonary venous connection can be made with confidence using transesophageal echocardiography (TEE). BACKGROUND: Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital anomaly whose diagnosis has classically been made using angiography. METHODS: We performed a retrospective review of all patients of all ages with PAPVC diagnosed at the Mayo Clinic who had undergone TEE because of either right ventricular volume overload or suspected intracardiac shunting by transthoracic echocardiography or intraoperatively. RESULTS: A total of 66 PAPVCs were detected in 43 patients (1.5/patient); in 2 additional patients, TEE suggested, but did not diagnose, PAPVCs. Shortness of breath was the most common presenting symptom (42.2%), followed by heart murmur and supraventricular tachycardia. Right-sided anomalous veins were identified in 35 patients (81.4%), left-sided in 7 (16.3%) and bilateral in 1 (2.3%). There was a single anomalous connecting vein in 23 patients (53.5%), two in 18 (41.9%), three in 1 (2.3%) and four in 1 (2.3%). The connecting site was the superior vena cava (SVC) in 39 veins (59.1%), right atrial-SVC junction in 6 (9.1%), right atrium in 8 (12.1%), inferior vena cava in 1 (1.5%) and the coronary sinus in 2 (3.0%). Ten anomalous left pulmonary veins were connected by a vertical vein to the innominate vein (15.1%). Sinus venosus atrial septal defect (ASD) was the most common associated anomaly in 22 patients (49%), followed by ostium secundum ASD in 6 and patent foramen ovale in 4. Fifteen patients had an intact atrial septum. Thirty-one patients (68.8%) underwent surgical repair. PAPVC was confirmed in all patients, including the two whose TEE results were suggestive of PAPVC. All 49 PAPVCs detected by TEE preoperatively were confirmed at the time of operation. CONCLUSIONS: TEE is highly diagnostic for PAPVC and can obviate angiography. Accurate anatomic diagnosis may influence the need for medical and surgical management. TEE should be performed in patients with right ventricular volume overload when the precordial examination is inconclusive.

Survival into adulthood of patients with unoperated single ventricle.

Adult patients with double-inlet left ventricle and perfectly balanced circulation may survive into the sixth decade with good functional capacity and preserved ventricular function. This should be considered before such patients are referred for a Fontan repair.

Calcified left ventricular mass: unusual clinical, echocardiographic, and computed tomographic findings of primary cardiac osteosarcoma.

Primary cardiac osteosarcomas are rare and usually originate in the left atrium. In contrast, osteosarcomas metastatic to the heart most commonly involve the right cardiac chambers. This case report describes an unusual primary cardiac osteosarcoma, initially observed as a slowly growing, densely calcified mass of the left ventricle with subsequent secondary pulmonary metastasis. Although cardiac tumors may be asymptomatic, this patient had recurrent bouts of ventricular tachyarrhythmia. We describe the clinical, echocardiographic, and radiological observations spanning 6 years and the gross and microscopic features at autopsy.

Transesophageal echocardiography: unusual case of anomalous pulmonary venous connection to the azygos vein.

Partial anomalous pulmonary venous connection, a rare congenital anomaly, most commonly involves the right lung, with one or more pulmonary veins anomalously connecting most frequently to the superior vena cava and less commonly to the right atrium or inferior vena cava. This article describes an unusual case of anomalous pulmonary venous connection of the right lung to the azygos vein in an adult. This anomaly was clearly delineated with angiography, computed tomography of the chest, and transesophageal echocardiography. The transesophageal echocardiographic features of the anomaly are described as a means to prevent further diagnostic misinterpretation.

Ventricular septal defects in adults.

Because congenital ventricular septal defects are of different sizes and locations, their clinical presentation, natural history, and treatment vary greatly. This review discusses the different types of ventricular septal defects commonly seen in adults in the authors' experience and in published literature. Ventricular septal defects are either isolated small defects or larger defects associated with pulmonary stenosis, pulmonary hypertension, or aortic regurgitation. These associations play an important role in the pathophysiologic consequences of the defect, its long-term complications, and treatment options. Knowledge of the different clinical presentations in adulthood and the specific features pertinent to these defects will help in the assessment and the care of adult patients with one of the most common congenital cardiac malformations.

An unusual presentation of atrial septal defect in a patient undergoing total hip arthroplasty.

IMPLICATIONS: Atrial septal defect is usually diagnosed and surgically repaired in childhood. We present a case of previously asymptomatic atrial septal defect that presented during total hip arthroplasty during general anesthesia in a 72-yr-old woman. Intraoperative transesophageal echocardiography assisted in the diagnosis and in determining the appropriate treatment.

Two-dimensional transesophageal echocardiographic determination of mitral valve area in adults with mitral stenosis.

Two-dimensional transthoracic echocardiography has been shown to be a reliable and accurate method of measuring stenotic mitral valve orifice area. Little data exist on the role of two-dimensional transesophageal echocardiography for this purpose. Thus in 45 adult patients with mitral stenosis mitral valve area was determined by direct planimetry with the use of two-dimensional transesophageal and transthoracic echocardiography. Transesophageal was less feasible than transthoracic echocardiography in the 45 patients (69% vs. 89%, p < 0.025). In 14 patients, two-dimensional transesophageal echocardiography was not feasible, primarily because of leaflet dropout. In 30 patients, transesophageal and transthoracic echocardiography were feasible, and measurements of mitral valve area by the two techniques correlated well (r = 0.91, SEE = 0.33 cm2, p < 0.0001). Mean mitral valve orifice area determined by transesophageal echocardiography (1.54 +/- 0.75 cm2; range 0.56 to 3.49 cm2) and by transthoracic echocardiography (1.55 +/- 0.78 cm2; range 0.62 to 3.68 cm2) did not differ (p = NS). The absolute (0.24 +/- 0.22 cm2) and percent (19% +/- 21%) differences between mitral valve area determined by transesophageal versus transthoracic echocardiography were small. These data show that mitral valve area in patients with mitral stenosis can be accurately measured by direct planimetry with two-dimensional transesophageal echocardiography. Technical refinements such as lateral-gain-compensation features may improve the feasibility of two-dimensional transesophageal echocardiography for measurements of mitral stenosis area, and this technique may become an adjunct to transthoracic echocardiography in the assessment of severity of mitral stenosis.

Endovascular stent implantation in a coronary artery to pulmonary artery fistula in a patient with pulmonary atresia with ventricular septal defect and severe cyanosis.

A 31-year-old male with pulmonary atresia, ventricular septal defect presented with exercise intolerance and severe cyanosis. A restrictive coronary-pulmonary artery fistula was identified as the main source of pulmonary blood flow. We report transcatheter stent implantation in the fistula to augment pulmonary flow as a palliative management option in the adult patient with complex congenital heart disease.

Mimics of Ebstein's anomaly.

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.

Dobutamine two-dimensional transesophageal echocardiographic stress testing for detection of coronary artery disease.

Atrial pacing and dipyridamole transesophageal echocardiography have been shown to be sensitive and specific tests for the detection of coronary artery disease. However, the sensitivity and specificity of dobutamine transesophageal echocardiography have not been reported. The purpose of this study was to determine the feasibility, sensitivity, and specificity of dobutamine transesophageal echocardiography for the detection of coronary artery disease. Transesophageal echocardiographic assessment of left ventricular function was performed in 81 adult patients aged 62 +/- 12 years during stepwise infusion of dobutamine from 5.0 to 40 micrograms/kg/min. Ischemia was diagnosed by the development of severe hypokinesis, akinesis, or dyskinesis of a previously contractile left ventricular segment. Coronary artery disease was defined by angiography as a reduction in luminal diameter of > or = 70% of an epicardial or > or = 50% of the left main coronary artery. In patients who had undergone coronary artery bypass graft surgery, a stenotic bypass graft was defined as a reduction in luminal diameter of > or = 70%. In patients without previous CABG, significant coronary artery disease was present in 21 patients: 5 with single-vessel disease, 7 double-vessel disease, 8 triple-vessel disease, and 1 left main coronary disease. Dobutamine transesophageal echocardiography had a sensitivity of 90% (19 of 21) and specificity of 94% (49 of 52) for the detection of coronary artery disease. In patients with previous CABG (n = 8), the sensitivity and specificity for the detection of bypass graft stenosis were 100% (4 of 4) and 75% (3 of 4), respectively.(ABSTRACT TRUNCATED AT 250 WORDS)