Emergency department recognition of acquired ventricular septal defect.

Acute myocardial infarction associated with ventricular septal defect (VSD) occurs infrequently. When a patient with an acquired VSD presents to the emergency department (ED), prompt recognition is required because definitive treatment can greatly decrease mortality. We present the case of a 75-year-old woman with an acute myocardial infarction and a new heart murmur. The diagnosis of acquired VSD was made by echocardiography in the ED, and emergency surgical correction was arranged.

The use of echocardiography in the emergency management of nonpenetrating traumatic cardiac rupture.

Three young adults, all drivers involved in three separate motor vehicle accidents that occurred in Portage County, Ohio, during a three-week period, sustained blunt, nonpenetrating chest trauma that resulted in right atrial rupture and cardiac tamponade. On arrival in the emergency department, all were hypotensive and complained of chest pain; only one had jugular venous distension and upper body cyanosis. All had normal chest radiographs, and one had ECG ST elevation. Echocardiography showed a moderate-sized pericardial effusion and signs of cardiac tamponade in all three. Two of the three survived emergency thoracotomy and repair of the right atrial rupture. These cases demonstrate the importance of echocardiography in diagnosing cardiac tamponade as the classic signs are not always present.

Thoracic cage deformities in the early diagnosis of the Marfan syndrome and cardiovascular disease.

The Marfan syndrome is frequently complicated by cardiovascular abnormalities. Of these, aortic dissection and aortic valve regurgitation are the most life-threatening. The most noticeable abnormalities of the Marfan syndrome--the skeletal abnormalities--may be subtle and limited. Presented here are five reports of cases of the Marfan syndrome. All patients had potentially lethal cardiovascular complications. Either the syndrome had not been previously diagnosed or the patient had not been adequately monitored despite the the presence of thoracic cage deformities present from youth. The purpose of this report is to heighten recognition of the association of thoracic cage deformities with the Marfan syndrome to permit early diagnosis of the associated cardiovascular complications. Surgical management of these complications can favorably alter the natural history of the Marfan syndrome.

Intraoperative Doppler color flow mapping for decision-making in valve repair for mitral regurgitation. Technique and results in 100 patients.

Mitral valve repair provides substantial advantages over mitral valve replacement in patients with severe mitral regurgitation. However, because of the possibility of persistent regurgitation, an intraoperative technique is needed to provide an immediate and accurate assessment of the adequacy of the repair before closure of the chest. One hundred patients with pure mitral regurgitation were studied with intraoperative epicardial Doppler color flow mapping immediately before and after valve repair. Intraoperative assessment of the severity of mitral regurgitation showed good agreement with preoperative left ventriculography and with standard precordial Doppler echocardiography before and after surgery. Postrepair intraoperative Doppler studies showed satisfactory surgical results in 92 patients. Postrepair intraoperative Doppler studies in the remaining eight patients demonstrated unsatisfactory results: persistent significant regurgitation in four, systolic anterior motion of the mitral valve with dynamic left ventricular outflow tract obstruction in three, and a persistent flail leaflet in one. In six of the eight patients, further surgery was performed during the same thoracotomy. In two patients, the intraoperative postrepair Doppler findings of persistent regurgitation were confirmed on precordial Doppler studies within 5 days, and mitral reoperation was required. Intraoperative epicardial Doppler color flow mapping provided a "safety net" that ensured a successful outcome in all 100 patients by providing the surgeon with a direct means to assess the success of the operation and the need for further surgery.

Reduction of intrapulmonary shunt and resolution of digital clubbing associated with primary biliary cirrhosis after liver transplantation.

This report describes a patient with marked hypoxemia caused by intrapulmonary shunt associated with primary biliary cirrhosis. Liver transplantation resulted in resolution of digital clubbing and reduction of intrapulmonary shunt as demonstrated by normalization of room air arterial blood gases, reduction in shunt fraction and normalization of the indocyanine-enhanced echocardiogram and perfusion lung scan. This patient's course challenges the conventional notion that intrapulmonary shunting associated with chronic liver disease does not reverse after liver transplantation.

Endomyocardial invasion by esophageal carcinoma. Demonstration by echocardiography.

Two cases of esophageal carcinoma with echocardiographically demonstrable direct extension are presented. Recognition of esophageal carcinoma metastatic to the heart is clinically difficult but can be facilitated by two-dimensional echocardiography. Earlier recognition of cardiac metastases by echocardiography may allow more effective therapy.

Precise evaluation of a complex coronary arteriovenous fistula: the utility of transesophageal color Doppler.

The transesophageal color flow Doppler appearance of coronary artery fistulas have not been described previously. We present a patient with a fistulous connection between the right coronary artery and the right atrium with an additional connection to the coronary sinus. The fistulous connection to the coronary sinus, confirmed at operation, was only diagnosed with transesophageal color flow Doppler and missed on both transthoracic echocardiography and cardiac catheterization. In addition, intraoperative transesophageal echocardiography was extremely useful in confirming closure of both these fistulas, thus confirming a successful operative result.

Pericardial disease: current diagnosis and management methods.

Diseases of the pericardium can be indolent or have a sudden onset. They may be primary, but more often are secondary to a systemic disease or previous therapy. Pericardial diseases imitate more common cardiac diseases and therefore can be difficult to diagnose. This paper's four case presentations illustrate use of recently developed methods that facilitate diagnosis. Magnetic resonance imaging is useful for the diagnosis of congenital absence of the pericardium. Echocardiography and computed tomography are useful for the diagnosis of pericardial cysts. Diagnosis of cardiac tamponade is aided by echocardiography, and Doppler echocardiography can help diagnose constrictive pericarditis. Effective management of pericardial diseases requires an understanding of the pathophysiology and natural history of each disease entity, knowledge of the individual patient, and realistic application of therapy.

Facial fuzz and funny findings. Facial hair causing otalgia and oropharyngeal pain.

Three patients with referred otalgia and/or oropharyngeal pain due to ectopic facial hair found in either the external auditory canal or oropharynx were treated at the Cleveland Clinic Foundation, Department of Otolaryngology and Communicative Disorders. In each of these patients, annoying symptoms were relieved by simple removal of the misplaced facial hair. Sensory nerve innervation of the external ear and oropharynx and their interrelationship in referred pain are described in detail following the case reports.

Long-term follow-up of patients with left ventricular outflow tract obstruction after Carpentier ring mitral valvuloplasty.

Left ventricular outflow tract (LVOT) obstruction is a complication of Carpentier ring mitral valvuloplasty that may occur only when this procedure is used to correct mitral regurgitation attributable to myxomatous degeneration of the mitral valve. LVOT obstruction has not been observed among approximately 300 patients undergoing this procedure to correct mitral regurgitation attributable to other causes. Among 200 patients with degenerative mitral regurgitation who underwent Carpentier valvuloplasty, LVOT obstruction was found in 12 patients (6%). Five of these patients demonstrated severe LVOT obstruction during intraoperative echocardiography immediately after repair, which was corrected by mitral valve replacement in four and removal of the Carpentier ring in one. The remaining seven patients were followed-up for a mean period of 27 months with history, physical examination, and Doppler echocardiography. Systolic anterior motion of the mitral valve was the echocardiographic hallmark of LVOT obstruction. Doppler echocardiographic and catheter-measured LVOT gradient paralleled the severity of the systolic anterior motion of the mitral valve. The severity of the motion decreased, but still could be provoked, with amyl nitrite at late follow-up. Mitral regurgitation tended to recur at late follow-up. Despite the presence of LVOT obstruction and hemodynamic features resembling hypertrophic cardiomyopathy at late follow-up, none of the patients had left ventricular hypertrophy or asymmetric septal hypertrophy, and early postoperative functional class improvement was sustained.

Clinical and angiographic features of coronary artery disease after chest irradiation.

Coronary artery disease (CAD) developed in 15 patients at a mean of 16 years (range 3 to 29) after chest irradiation. The mean dose of radiation was 42 +/- 7 grays; irradiation was performed for Hodgkin's disease in 9 patients, lymphoma in 2, breast carcinoma in 3 and cystic hygroma in 1 patient. Mean age was 48 years (range 26 to 63) at diagnosis of CAD; 4 patients were younger than 35 years. Nine were women. Ten presented with angina, 3 with acute myocardial infarction, 1 patient with syncope and 1 with dyspnea. Twelve had no more than 2 risk factors of atherosclerosis. At coronary angiography, 8 had at least 50% diameter narrowing of the left main coronary artery and 4 had severe ostial stenosis of the right coronary artery. Eight patients also had valvular heart disease, 4 pericardial disease and 4 complete heart block. Mean left ventricular ejection fraction was 67 +/- 11% (range 53 to 80%). Nine had undergone coronary artery bypass grafting, but surgery was difficult or impossible in 3 because of severe mediastinal and pericardial fibrosis. Radiation-associated CAD is characterized by a high incidence of left main and right ostial coronary disease and often occurs in women with relatively few conventional risk factors for CAD.

Evaluation of normal prosthetic valve function by Doppler echocardiography.

Previous investigations have suggested that Doppler echocardiography is useful in detecting dysfunction in aortic (AVR) and mitral prostheses (MVR). However, to diagnose abnormalities, the spectrum of normal velocities through these valves must be established. Therefore, we used Doppler echocardiography to study 100 patients with 105 prosthetic valves that had no clinical evidence of valve dysfunction 9 +/- 8 days postoperatively. There were 66 Carpentier-Edwards (C-E), 23 St. Jude (S-J), and 16 Ionescu-Shiley (I-S) valves. In 70 AVR, the peak instantaneous gradient was 26.4 +/- 8.2 Hg, mean systolic gradient was 15.6 +/- 5 mm Hg, and gradients varied inversely with valve size, although differences were significant only when comparing the smallest vs the largest valve sizes (p less than or equal to 0.03). Peak instantaneous gradients greater than 36 mm Hg occurred only in AVR size 23 or smaller. There were no significant differences in gradients among C-E, S-J, and I-S AVR. In 35 MVR, mean gradient was 6.9 +/- 2.3 mm Hg and valve area was 2.7 +/- 0.8 cm2; neither varied significantly with valve size. However, S-J MVR group had smaller mean gradients and larger effective valve area than C-E bioprosthetic MVR (p = 0.01 and p = 0.05, respectively). Regurgitation was more common in AVR (26%) than in MVR (9%), p = 0.04, although all instances were mild and clinically silent. We conclude that normal AVR and MVR of a given size and type have a predictable range of Doppler echocardiographic parameters. Doppler evidence of mild regurgitation is a frequent finding in normal AVR and MVR.(ABSTRACT TRUNCATED AT 250 WORDS)

Intraoperative Doppler echocardiography in hypertrophic cardiomyopathy: correlations with the obstructive gradient.

Although significant pressure gradients can be recorded across the left ventricular outflow tract in patients with hypertrophic cardiomyopathy, controversy exists regarding the presence or absence of true obstruction. Ten patients with hypertrophic cardiomyopathy were studied at the time of septal myectomy. A sterile continuous wave Doppler transducer was placed on the ascending aorta and directed toward the left ventricular outflow tract to measure velocity simultaneously with invasive gradient measured using solid-state hub transducers by direct puncture of the left ventricle and aorta. Simultaneous Doppler velocity and invasive gradient measurements (n = 33) were made at rest, before and after myectomy and during interventions with isoproterenol, volume loading and phenylephrine. High velocity flow with a characteristic contour was recorded in patients with a significant gradient. Using the modified Bernoulli equation (gradient = 4 X velocity), a good correlation was found between the Doppler-derived gradient and the peak instantaneous gradient measured invasively (r = 0.93, y = 0.89X + 12, p = 0.0001). Changes in gradient and velocity due to interventions also correlated well (r = 0.96, y = 0.91X - 3, p = 0.0001). Continuous wave Doppler echocardiography can accurately estimate the outflow tract gradient. The magnitude, timing and contour of these high velocity flow signals support the hypothesis that true obstruction is present in patients with hypertrophic cardiomyopathy who have a significant gradient.