Quadricuspid Aortic Valvulopathy and Acute Type A Aortic Dissection.

This case report describes a 55-year-old male who presented with acute Type A aortic dissection. He underwent emergent surgical repair, and his intraoperative transesophageal echocardiography revealed a quadricuspid aortic valve. His aortic root measured 45 mm. Quadricuspid aortic valves have previously been associated with aortic root dilation. This case illustrates the possible association of quadricuspid aortic valves with aortic dissection, similar to what is described with bicuspid valves.

False positive computed tomographic angiography for Stanford type A aortic dissection.

BACKGROUND: Computed tomographic angiography (CTA) has emerged as the defacto imaging test to rule out acute aortic dissection; however, it is not without flaws. We report a case of a false-positive CTA with respect to Stanford Type A aortic dissection. CASE: A 52 year-old male presented with sudden onset shortness of breath. He denied chest pain. Due to severe hypertension and an Emergency Department bedside ultrasound suggesting an intimal flap in the aorta, CTA was requested to better assess the ascending aorta and was interpreted as consistent with Stanford Type A aortic dissection with thrombosis of the false lumen in the ascending aorta. However, intra-operative imaging (TEE and epi-aortic scanning) did not identify an intimal flap or dissection, and neither did definitive surgical inspection of the aorta. The suspected aortic dissection and thrombosed false lumen were not visualized on repeat CTA two days later. DISCUSSION: False positive diagnosis of Stanford Type A aortic dissection on CTA can be the result of technical factors, streak artifacts, motion artifacts, and periaortic structures. In this case, non-uniform arterial contrast enhancement secondary to unrecognized biventricular dysfunction resulted in the false positive CTA appearance of an intimal flap and mural thrombus. Intra-operative TEE and epi-aortic scanning were proven correct in excluding aortic dissection by the standard of definitive surgical inspection of the aorta.

Unique congenital malformation of the mitral valve associated with anomalous coronary arteries and stroke.

A 55-year-old male presented with stroke. Transesophageal echocardiogram and cardiac computed tomography revealed an unrecognized congenital malformation of the anterior mitral leaflet associated with anomalous left coronary circumflex artery, arising from the right coronary artery, diagnosed first by echocardiogram. This case represents a unique unforeseen mitral valve anomaly that might be considered as potential cardiac source of embolism. This finding broadens the spectrum of known mitral valve anomalies.

Thoracic aortic frontier: review of current applications and directions of thoracic endovascular aortic repair (TEVAR).

Thoracic endovascular aortic repair, a minimally invasive technique is replacing the maximally invasive gold standard of thoracotomy and replacement of the descending thoracic aorta. With experience, indications have expanded to encroach on the arch and even ascending aorta. This review highlights the current state of technology, discusses controversies, and takes the perspective of a forward-thinking review to describe novel, innovative techniques that might make the entire thoracic aorta amenable to minimally invasive repair.

Painless Type B Aortic Dissection: Insights From the International Registry of Acute Aortic Dissection.

INTRODUCTION: The classical presentation of a patient with Type B acute aortic dissection (TBAAD) is characterized by severe chest, back, or abdominal pain, ripping or tearing in nature. However, some patients present with painless acute aortic dissection, which can lead to a delay in diagnosis and treatment. We utilized the International Registry on Acute Aortic Dissections (IRAD) database to study these patients. METHODS: We analyzed 43 painless TBAAD patients enrolled in the database between January 1996 and July 2012. The differences in presentation, diagnostics, management, and outcome were compared with patients presenting with painful TBAAD. RESULTS: Among the 1162 TBAAD patients enrolled in IRAD, 43 patients presented with painless TBAAD (3.7%). The mean age of patients with painless TBAAD was significantly higher than normal TBAAD patients (69.2 versus 63.3 years, P = 0.020). The presence of atherosclerosis (46.4% versus 30.1%, P = 0.022), diabetes (17.9% versus 7.5%; P = 0.018), and other aortic diseases (8.6% versus 2.3%, P= 0.051), such as prior aortic aneurysm (31% versus 18.8% P = 0.049) was more common in these patients. Median delay time between presentation and diagnosis was longer in painless patients (median 34.0 versus 19.0 hours; P = 0.006). Dissection of iatrogenic origin (19.5% versus 1.3%; P < 0.001) was significantly more frequent in the painless group. The in-hospital mortality was 18.6% in the painless group, compared with an in-hospital mortality of 9.9% in the control group (P = 0.063). CONCLUSION: Painless TBAAD is a relatively rare presentation (3.7%) of aortic dissection, and is often associated with a history of atherosclerosis, diabetes, prior aortic disease including aortic aneurysm, and an iatrogenic origin. We observed a trend for increased in-hospital mortality in painless TBAAD patients, which may be the result of a delay in diagnosis and management. Therefore, physicians should be aware of this relative rare presentation of TBAAD.

Titration and implementation of neurally adjusted ventilatory assist in critically ill patients.

BACKGROUND: Neurally adjusted ventilatory assist (NAVA) delivers assist in proportion to the patient's respiratory drive as reflected by the diaphragm electrical activity (EAdi). We examined to what extent NAVA can unload inspiratory muscles, and whether unloading is sustainable when implementing a NAVA level identified as adequate (NAVAal) during a titration procedure. METHODS: Fifteen adult, critically ill patients with a Pao(2)/fraction of inspired oxygen (Fio(2)) ratio < 300 mm Hg were studied. NAVAal was identified based on the change from a steep increase to a less steep increase in airway pressure (Paw) and tidal volume (Vt) in response to systematically increasing the NAVA level from low (NAVAlow) to high (NAVAhigh). NAVAal was implemented for 3 h. RESULTS: At NAVAal, the median esophageal pressure time product (PTPes) and EAdi values were reduced by 47% of NAVAlow (quartiles, 16 to 69% of NAVAlow) and 18% of NAVAlow (quartiles, 15 to 26% of NAVAlow), respectively. At NAVAhigh, PTPes and EAdi values were reduced by 74% of NAVAlow (quartiles, 56 to 86% of NAVAlow) and 36% of NAVAlow (quartiles, 21 to 51% of NAVAlow; p < or = 0.005 for all). Parameters during 3 h on NAVAal were not different from parameters during titration at NAVAal, and were as follows: Vt, 5.9 mL/kg predicted body weight (PBW) [quartiles, 5.4 to 7.2 mL/kg PBW]; respiratory rate (RR), 29 breaths/min (quartiles, 22 to 33 breaths/min); mean inspiratory Paw, 16 cm H(2)O (quartiles, 13 to 20 cm H(2)O); PTPes, 45% of NAVAlow (quartiles, 28 to 57% of NAVAlow); and EAdi, 76% of NAVAlow (quartiles, 63 to 89% of NAVAlow). Pao(2)/Fio(2) ratio, Paco(2), and cardiac performance during NAVAal were unchanged, while Paw and Vt were lower, and RR was higher when compared to conventional ventilation before implementing NAVAal. CONCLUSIONS: Systematically increasing the NAVA level reduces respiratory drive, unloads respiratory muscles, and offers a method to determine an assist level that results in sustained unloading, low Vt, and stable cardiopulmonary function when implemented for 3 h.

Mitral stenosis and cardiogenic shock from an obstructive abscess of the mitral annulus.

A 48-year-old female hemodialysis patient with gram-positive mitral endocarditis developed progressive hemodynamic collapse. Echocardiography revealed a mitral annular abscess, which expanded rapidly due to connection with and pressure from the left ventricle. The mitral annular abscess had enlarged sufficiently to obstruct the mitral orifice, resulting in acute cardiogenic shock. The patient underwent and survived emergency surgical repair, and remained well on follow-up.

Saphenous vein graft aneurysm masquerading as a left atrial mass: diagnosis by contrast transesophageal echocardiography.

We describe the first reported case of a saphenous vein graft aneurysm mimicking a left atrial (LA) mass on echocardiographic imaging in a 62-year-old man 22 years after coronary bypass surgery. On presentation with worsening angina, a transthoracic echocardiogram revealed a LA mass. Coronary angiography showed severe native coronary artery and graft disease. The saphenous vein graft to the first obtuse marginal branch of the left circumflex coronary artery appeared ectatic and aneurysmal and was diffusely diseased. Transesophageal echocardiography demonstrated a well-circumscribed mass apparently within the LA, with an area of central echolucency and a surrounding crescentic region of increased echodensity, with absent flow by color and spectral Doppler techniques. After an intravenous bolus injection of Definity, the area of central echolucency within the mass was opacified compatible with a vascular origin. Pulsatile contrast flow was seen predominantly in diastole consistent with a coronary arterial flow pattern. Thus, contrast transesophageal echocardiography confirmed the diagnosis of a saphenous vein graft aneurysm invaginating the lateral aspect of the LA, mimicking a true LA mass, highlighting a novel use for this technology.

Frequency of and inappropriate treatment of misdiagnosis of acute aortic dissection.

Acute aortic syndrome (AAS) comprises acute aortic dissection, intramural hematoma, and penetrating ulcer of the aorta. The importance of accurate, rapid diagnosis and intervention for AAS is underscored by its clinical and epidemiologic overlap with acute coronary syndrome and by the risks of inappropriate treatment with antithrombotic agents. To explore these concerns, the recognition, management, and outcomes of AAS in the contemporary experience of a tertiary referral center were reviewed. Sixty-six consecutive patients with AAS admitted from January 2000 to December 2004 were identified, and their records reviewed. Misdiagnosis occurred in 39% (n = 26) and was associated with longer time to correct diagnosis (mean +/- SEM 51 +/- 12 vs 15 +/- 5 hours, p = 0.003). Acute coronary syndrome was the most common misdiagnosis, resulting in inappropriate treatment with acetylsalicylic acid in 26 (100%), clopidogrel in 1 (4%), heparin in 22 (85%), and fibrinolytic agents in 3 (12%). Exposure to antithrombotic agents was associated with higher rates of major bleeding (38% vs 13%) and a trend toward greater in-hospital mortality (27% vs 13%) (p = 0.02 for combined end point). Antithrombotic agent administration was also associated with increased hemorrhagic pericardial fluid (50% vs 25%), hemorrhagic pleural effusion (15% vs 3%), and hemodynamic instability (30% vs 13%) (p = 0.02 for combined end point). In conclusion, AAS is frequently confused with acute coronary syndrome, leading to delayed diagnosis and clinically significant bleeding as a consequence of inappropriate treatment with antithrombotic agents.

Impact of radial artery cannulation for coronary angiography and angioplasty on radial artery function.

The radial artery is commonly used as a conduit in coronary artery bypass grafting. No data exist on the effects of radial sheath insertion on radial artery function. Because many patients considered for coronary artery bypass grafting have had previous radial procedures, it is important to understand any effects radial sheath insertion may have on radial artery function. Twenty-two patients who underwent elective coronary angiography or angioplasty with a 6Fr sheath through the right radial artery were studied. Radial artery function was assessed using ultrasound to measure flow-mediated dilation (FMD). Reactive hyperemia was produced by 5-minute cuff inflation on the arm to suprasystolic pressures. Radial artery diameter was measured at rest and 1 minute after cuff deflation. FMD was expressed as percent change in radial diameter compared with at rest. In all cases, the left radial artery was studied as a control. Patients were studied before sheath insertion, immediately after sheath insertion, and 6 weeks after sheath insertion. The FMD of the cannulated arm was 13.2% before sheath insertion versus 3.6% immediately after sheath insertion (p <0.01) and 0.2% (p <0.01) 9 weeks after sheath insertion. In contrast, there were no significant changes in the noncannulated arm at either time point. In conclusion, radial artery sheath insertion for coronary angiography or angioplasty results in immediate and persistent blunting of FMD, suggesting severe vasomotor dysfunction. Radial artery sheath insertion has important effects on radial artery function that must be considered when selecting radial conduits for coronary artery bypass grafting.

Dehydroepiandrosterone sulfate induces acute vasodilation of porcine coronary arteries in vitro and in vivo.

Although an inverse relationship between dehydroepiandrosterone sulfate (DHEAS) and coronary artery disease has been demonstrated in men, the vascular effects of DHEAS are not well defined. The vasoactive effects of intracoronary DHEAS and testosterone (0.1 nM to 1 microM) were examined in vivo in 24 pigs. Epicardial cross-sectional area was measured by intravascular ultrasound, and coronary flow velocity by intravascular Doppler velocimetry. We also examined the effects of antagonism of the androgen receptor, nitric oxide synthase, and potassium channels on DHEAS-induced vasodilation in vitro in coronary rings from male and female pig hearts. DHEAS and testosterone induced increases in cross-sectional area, average peak velocity, and coronary blood flow. The maximal increase in coronary blood flow in response to testosterone was 1.26-fold (P=0.02), and in average peak velocity 1.43-fold (P=0.05), greater than that to DHEAS, whereas increases in cross-sectional area were similar. Vasodilation to both hormones was rapid, with maximal responses occurring <10 minutes after administration. In vitro, DHEAS and testosterone induced vasodilation in coronary rings, greater with testosterone. At doses of 0.1 and 1 microM, the vasodilator effects of DHEAS and testosterone were inhibited by the androgen receptor antagonist flutamide but not the estrogen receptor antagonist ICI 182,780. At 10 microM, neither DHEAS- nor testosterone-induced vasorelaxation was inhibited by flutamide, ICI 182,780, L-NAME, or deendothelialization, but both were attenuated by pretreatment with glibenclamide. No gender differences were observed in any of the responses examined. In conclusion, DHEAS is an acute coronary artery vasodilator, but less potent than testosterone. Its effect might be mediated via androgen receptors and may involve ATP-sensitive potassium channels.

Prosthetic valve thrombosis after initiation of hormone replacement therapy.

The complications of hormone replacement therapy (HRT) related to hypercoagulability are well known. However, there have been no cases of prosthetic valve thrombosis reported in the literature in conjunction with HRT. The present report discusses a case of acute mitral prosthetic valve thrombosis associated with the initiation of HRT. Because hypercoagulability is usually associated with significant morbidity and mortality, caution should be exercised in the management of patients with prosthetic valve thrombosis receiving HRT.

Transthoracic echocardiography to identify or exclude cardiac cause of shock.

BACKGROUND: Transesophageal echocardiography (TEE) is often still considered the echocardiographic test of choice in the general ICU patient population to establish the presence or absence of cardiac cause of shock, and is often requested and performed as the initial and only echocardiographic test. This premise is based on older studies in which transthoracic echocardiography (TTE) commonly offered inadequate images in ICU patients. STUDY OBJECTIVES: We hypothesized that current TTE imaging alone is adequate to identify or exclude cardiac cause of shock in the great majority of cases. METHODS: One hundred consecutive shock cases in which an echocardiogram was requested were prospectively analyzed by two blinded echocardiographers for image adequacy, and the absence or presence of cardiac cause of shock (defined as one or more of the following: severe left ventricular (LV) or right ventricular systolic dysfunction, tamponade, severe left-sided valve disease, or a postinfarction mechanical complication), and compared to a clinical standard of presence/absence of cardiac cause of shock as determined by autopsy, surgery, or objective testing. Shock was defined as systolic BP < 100 mm Hg or fall in BP >/= 25%, and inotrope use or evidence of low output or venous congestion. Cardiac output was determined by the LV outflow tract (LVOT) Doppler method. RESULTS: Sixty-three percent of cases had a cardiac cause of shock. TTE image quality was adequate in 99% cases. Among the 99% of cases in which the imaging was adequate, the sensitivity of TTE for cardiac cause of shock was 100%, the specificity was 95%, the positive predictive value was 97%, and the negative predictive value was 100%. There were relative contraindications to TEE in 15% of cases. Stroke volume index (15 +/- 6 mL/m(2) vs 31 +/- 7 mL/m(2) [mean +/- 1 SD]; p < 0.001) and cardiac index (1.6 +/- 0.5 mL/min/m(2) vs 2.9 +/- 0.9 mL/min/m(2); p < 0.001) were significantly less in the group with a cardiac cause of shock than in the group with a noncardiac cause of shock. CONCLUSIONS: In the general critical care population, current TTE imaging identifies the great majority of cardiac causes of shock. TTE should be considered not only the initial, but also the principal echocardiographic test in the critical care environment.

Contrast echocardiography remains positive after treatment of pulmonary arteriovenous malformations.

STUDY OBJECTIVES: Pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT) can cause hemorrhage, stroke, and cerebral abscess. Therapy consists of transcatheter embolotherapy (TCET) to occlude the PAVMs. Contrast transthoracic echocardiography (TTE) can be used to screen for PAVMs, but little is known about the performance of contrast TTE after TCET has been performed. Our objective was to determine the effect of the successful performance of TCET on the performance of contrast TTE, specifically, in what proportion of patients the findings of contrast TTE normalized or remained positive after the performance of TCET. DESIGN: Retrospective chart review. SETTING: HHT clinic at university teaching hospital. PATIENTS: Patients who have undergone TCET for the treatment of PAVMs. INTERVENTIONS: Patients were screened for PAVMs with a chest radiograph (CXR), oxygen shunt test (OST), and contrast TTE. Pulmonary angiography was recommended for patients with any positive findings on a screening test. PAVMs > or = 3 mm were occluded by TCET. Contrast TTE, OST, and CXR were performed approximately 1 month later. The results of contrast TTE before and after patients underwent TCET were compared. MEASUREMENTS AND RESULTS: Thirty-nine patients underwent contrast TTE prior to undergoing TCET, and 29 patients underwent contrast TTE both prior to and after undergoing TCET. In all patients, TTE findings were positive prior to TCET. All PAVMs with feeding vessels > or = 3 mm were successfully occluded based on completion angiography. After TCET, 48% of patients had no detectable residual PAVMs, and the remainder had small (ie, < 3 mm) residual PAVMs. Of the 29 patients, 90% had positive contrast TTE findings after undergoing TCET. In the subset of patients who had no residual PAVMs on the completion angiography, 80% had positive contrast TTE findings after undergoing TCET. CONCLUSIONS: In most patients, contrast TTE findings remain positive after they undergo TCET, even in patients without residual PAVMs seen on angiography. This may reflect residual PAVMs that are too small to visualize using angiography. These findings have important implications for the follow-up and management of HHT patients.

Cardiac encasement by metastatic myxoid liposarcoma.

Isolated cardiac metastasis from a primary liposarcoma of noncardiac origin is a rare occurrence. A patient who presented with biventricular failure and constrictive hemodynamics years after successful resection of a primary liposarcoma of the thigh is described. Extensive cardiac encasement by tumor was suspected on diagnostic imaging. Hemodynamic instability and multiorgan failure necessitated urgent exploratory sternotomy. The patient died intraoperatively. Extensive metastatic sarcoma limited to the heart was confirmed during surgical procedure. This case suggests that in clinical and pathological investigation of a cardiac mass, knowledge of previous extracardiac involvement with soft tissue sarcoma is essential.

The 'wet Westermark' sign.

A 67-year-old woman experienced dyspnea and exertional intolerance after aortocoronary bypass grafting. Early in the postoperative course, she had mild heart failure due to volume overload. Regional oligemia was appreciated on the chest radiograph. The oligemia led to the consideration of pulmonary embolism and was corroborated by perfusion scanning, which demonstrated defects in the same areas. Interstitial markings, accentuated by the heart failure, served as a serendipitous form of radiographic 'contrast' - a marker of blood perfusion and, in the present case, regional underperfusion. Therefore, mild heart failure accentuates 'Westermark's' sign.

Right heart obstruction from a balloon-like sinus of Valsalva aneurysm in a patient with Down syndrome.

A 40-year-old man with Down syndrome presented with right heart failure. He was markedly obese and had severe developmental delay. There was marked edema and an early diastolic sound. Transthoracic echocardiography suggested a right heart mass. Transesophageal echocardiography revealed an unruptured balloon-like sinus of Valsalva aneurysm within the right atrium that obstructed the tricuspid orifice. The patient died in hospital of mixed obstructive and/or septic shock.

Transesophageal Echocardiographic Assessment of Lesions of the Right Ventricular Outflow Tract and Pulmonic Valve.

To establish the role of biplane transesophageal echocardiography (TEE) in the assessment of congenital and acquired lesions involving the right ventricular outflow tract (RVOT) and pulmonic valve (PV), 28 consecutive RVOT and PV lesions in 22 consecutive patients were studied by two-dimensional and color Doppler transthoracic echocardiograms (n = 22), horizontal (n = 22) and vertical (n = 22) plane TEEs, cardiac catheterization (n = 15), cardiac surgery (n = 6), and magnetic resonance imaging (n = 1). Sixteen patients had congenital lesions, and six had acquired lesions. Longitudinal TEE clearly imaged 25 of 28 abnormalities, transverse TEE clearly imaged 12 of 28, and transthoracic echocardiography clearly imaged 9 of 28. Two-dimensional TEE scanning revealed the lesion or site of stenosis. Color Doppler revealed conspicuous mosaic jets in relation to a structural abnormality in most cases. Longitudinal TEE was more sensitive in the detection of small vegetations of the PV, in the depiction of PV doming in cases of valvar pulmonic stenosis, and in the display of the RVOT and PV so that the longitudinal extent of involvement of larger masses could be appreciated. However, longitudinal TEE was not able to assess the gradient of a stenosis at the RVOT or PV level in any case. Biplane TEE is helpful in the anatomic assessment of congenital and acquired lesions of the RVOT and PV in adults. (ECHOCARDIOGRAPHY, Volume 13, January 1996)