Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease with many genotype and phenotype variations. Earlier terminologies, hypertrophic obstructive cardiomyopathy and idiopathic hypertrophic sub-aortic stenosis are no longer used to describe this entity. Patients present with or without left ventricular outflow tract (LVOT) obstruction. Resting or provocative LVOT obstruction occurs in 70% of patients and is the most common cause of heart failure. The pathology and pathophysiology of HCM includes hypertrophy of the left ventricle with or without right ventricular hypertrophy, systolic anterior motion of mitral valve, dynamic and mechanical LVOT obstruction, mitral regurgitation, diastolic dysfunction, myocardial ischemia, and fibrosis. Thorough understanding of pathology and pathophysiology is important for anesthetic and surgical management.

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A 76-year-old female patient presented for an endoscopic retrograde cholangiopancreatography (ERCP) for the removal of a biliary stent and lithotripsy. During the procedure, an acute drop in the end-tidal CO ₂ , followed by cardiovascular collapse prompted the initiation of the advanced cardiac life support protocol. Transesophageal echocardiography (TEE) demonstrated direct evidence of pulmonary embolism. The patient was promptly treated with thrombolytic therapy and subsequently discharged home on oral warfarin therapy, with no noted sequelae. Although, there have been case reports of air embolism during an ERCP presenting with cardiovascular collapse, to the best of our knowledge, there are no reported cases of acute pulmonary embolus during this procedure. While the availability of TEE in the operating suites is quite common, quick access and interpretation capabilities in remote locations may not be as common. With the expansion of anesthesia services outside of the operating rooms, it may be prudent to develop rapid response systems that incorporate resources such as TEE and trained personnel to deal with such emergent situations.

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Improved cosmetic appearance, reduced pain and duration of post-operative stay have intensified the popularity of minimally invasive cardiac surgery (MICS); however, the increased risk of stroke remains a concern. In conventional cardiac surgery, surgeons can visualize and feel the cardiac structures directly, which is not possible with MICS. Transesophageal echocardiography (TEE) is essential during MICS in detecting problems that require immediate correction. Comprehensive evaluation of the cardiac structures and function helps in the confirmation of not only the definitive diagnosis, but also the success of surgical treatment. Venous and aortic cannulations are not under the direct vision of the surgeon and appropriate positioning of the cannulae is not possible during MICS without the aid of TEE. Intra-operative TEE helps in the navigation of the guide wire and correct placement of the cannulae and allows real-time assessment of valvular pathologies, ventricular filling, ventricular function, intracardiac air, weaning from cardiopulmonary bypass and adequacy of the surgical procedure. Early detection of perioperative complications by TEE potentially enhances the post-operative outcome of patients managed with MICS.

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A 36-year-old male patient presented with the complaints of palpitations and breathlessness. Preoperative transthoracic echocardiography (TTE) revealed a bicuspid aortic valve; severe aortic regurgitation with dilated left ventricle (LV) and mild LV systolic dysfunction (ejection fraction 50%). He was scheduled to undergo aortic valve replacement. History was not suggestive of infective endocarditis (IE). Preoperative TTE did not demonstrate any aortic perivalvular abscess. Intraoperative transesophageal echocardiography (TEE) examination using the mid-esophageal (ME) long-axis view, showed an abscess cavity affecting the aortic valve, which initially was assumed to be a dissection flap, but later confirmed to be an abscess cavity by color Doppler examination. The ME aortic valve short-axis view showed two abscesses; one was at the junction of the non-coronary and left coronary commissure and the other one above the right coronary cusp. Intraoperatively, these findings were confirmed by the surgeons. The case report demonstrates the superiority of TEE over TTE in diagnosing perivalvular abscesses.

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Takotsubo cardiomyopathy also known as transient apical ballooning syndrome or stress induced reversible cardiomyopathy is an increasingly reported syndrome generally characterized by transient systolic dysfunction of the apical and/or mid segment of the left ventricle. It is frequently precipitated by severe stress and clinically mimics an acute ST-elevation myocardial infarction, with angiographically normal coronary arteries. A high index of suspicion is needed to diagnose this syndrome. We describe a patient who developed Takotsubo cardiomyopathy in the post-operative period following vaginal hysterectomy.

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Aims and Objectives: Perioperative optic neuropathy (PON) is a rare, but devastating complication following coronary artery bypass graft surgery (CABG). We performed a retrospective study of PON associated with off-pump CABG (OPCABG) to identify possible risk factors. Materials and Methods: 1442 patients underwent OPCABG over a 10-month period from October 2008 to August 2009; PON was identified in four (0.28%) patients. A retrospective review of the charts was done to identify the patient characteristics, pre-operative status, intra-operative details, and ophthalmic examination details. Friedman test was used to compare the hematocrit (Hct) and the mean arterial pressure (MAP) values across the three time periods: Pre-, intra- and post-operative periods. Results: All four patients were male, diabetic, and in the age range 51-69 years. All patients noted unilateral or bilateral severe visual loss in the immediate post-operative period, which was permanent. All the four patients had statistically significant decrease in the Hct (P < 0.039) and mean arterial blood pressure (P < 0.018) in the intraoperative and post-operative period when compared to pre-operative value. Conclusions : PON is a rare but definite possibility in patients undergoing OPCABG. Diabetes mellitus may be a risk factor. Perioperative hemodynamic abnormalities like decrease in MAP and anemia may play a role in the development of PON in OPCABG.

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We describe postoperative refractory ventricular tachycardia (VT) in a patient following aortic and mitral valve replacement. Following an uneventful separation from cardiopulmonary bypass with dobutamine, the patient developed recurrent VT, 4 hours into the postoperative period. The VT did not respond to standard doses of xylocard, magnesium and amiodarone. Electrolyte and acid base parameters were normal. Multiple cardioversions failed to revert back to a stable rhythm. Intra-aortic balloon pumping was instituted and overdrive right ventricular pacing was unsuccessful. Following intravenous sotalol 80 mg, the VT came under control and reverted to a nodal rhythm, which required atrial pacing for the next 8 hours. Oral sotalol therapy was continued at 40 mg daily. The VT did not recur.

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A 3-month-old male child underwent uneventful inguinal herniotomy under general anesthesia. After extubation, airway obstruction followed by pulmonary edema appeared for which the baby was reintubated and ventilated. The baby made a complete recovery and extubated after about 2 h. A post-operative computed tomography scan revealed a posterior mediastinal cystic mass abutting the tracheal bifurcation. Presumably, extrinsic compression by the mass on the tracheal bifurcation led to the development of negative pressure pulmonary edema.

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Aorto-atrial fistula is a rare complication of prosthetic aortic valve replacement (AVR) and most of them have been diagnosed as a late complication. We present a case of this unusual complication after AVR. Intraoperative transoesophageal echocardiography identified and diagnosed this rare and potentially disastrous surgical complication and confirmed adequacy of its surgical repair.

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Aims and Objectives: Cor triatriatum sinistrum (CTS) and cor triatriatum dextrum (CTD) are rare congenital anomalies characterized by the presence of a perforated septum which divides the respective atrium into a proximal and distal chamber. This report reviews the perioperative course of patients with uncorrected cor triatriatum (CT) undergoing procedures requiring anesthesia. In addition, we performed a literature search that examines the experience of others regarding the peri-operative course of patients with CT. Materials and Methods: A computerized search of a medical record database was conducted to identify patients with a clinical diagnosis of uncorrected CTD and CTS undergoing surgical procedures. Descriptive statistics were used. Results: We identified 12 adult patients with asymptomatic CTS (n = 7) and CTD (n = 5) who underwent 23 anesthetics. There were no perioperative complications which could be attributed directly to the anatomy of CT. Conclusions: Our observation and review of the literature suggest that patients with asymptomatic CT typically tolerate anesthesia and surgical procedures well.

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Mitral regurgitation is uncommon with left atrial myxoma. The echocardiographic assessment of presence of mitral regurgitation and its severity are impaired by the presence of left atrial myxoma. We describe an uncommon association of left atrial myxoma with coronary artery disease and mitral regurgitation. MR was reported as mild on pre-operative transthoracic echocardiography but found to be severe due to ruptured chordae tendinae during intra-operative transesophageal echocardiography, which lead to change in the surgical plan to mitral valve replacement in addition to excision of myxoma.

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A 62 year old female with severe mitral stenosis, large left atrial ball thrombus and acute mesenteric ischemia emergently underwent mitral valve replacement, left atrial clot removal and emergency laparotomy for mesenteric ischemia. Peri-operative management issues, particularly, the anesthetic challenges and the role of transesophageal echocardiography are discussed.

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Pseudoaneurysm of mitral-aortic intervalvular fibrosa (P-MAIVF) is a rare cardiac surgical condition. P-MAIVF commonly occurs as a complication of aortic and mitral valve replacement surgeries. The surgical trauma during replacement of the valves weakens the avascular mitral and aortic intervalvular area. We present a case of P-MAIVF recurrence 5 years after a primary repair. Congestive cardiac failure was the presenting feature with mitral and aortic regurgitation. In view of the recurrence, the surgical team planned for a double valve replacement. The sewing rings of the two prosthetic-valves were interposed to close the mouth of the pseudoaneurysm and to provide mechanical reinforcement of the MAIVF. Intra-operative transesophageal echocardiography (TEE) helped in delineating the anatomy, extent of the lesion, rupture of one of the pseudoaneurysm into left atrium and severity of the valvular regurgitation. Post-procedure TEE confirmed complete obliteration of the pseudoaneurysm and prosthetic valve function.

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Endobronchial spillage of fungal material into normal lung can infect it and the spillage of fungal material should be prevented during surgery. We report our experience of a patient who presented for right upper lobectomy with bronchiectasis, tubercular destruction and subsequent aspergilloma. A 4F Fogarty catheter was introduced through the tracheal lumen of the left sided endobronchial double lumen tube (DLT) to occlude the bronchus intermedius to prevent spillage of aspergilloma into the non-infected lower and middle lobes of the right lung. The Fogarty catheter was pulled into the trachea just before stapling the bronchus; thereafter, right upper lobectomy was completed successfully. The patient was extubated uneventfully and transferred to post-operative recovery ward. The endobronchial blockage of the intermediate bronchus of the operative lung by the Fogarty catheter and isolation of the left lung by the DLT prevented spillage of aspergilloma in both the operative right lung and the left lung.

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Nitric oxide (NO) is a selective pulmonary vasodilator especially in the presence of pulmonary artery hypertension. With right ventricle (RV) dysfunction, inhaled NO may increase RV ejection fraction and cardiac output. The main advantage of NO over intravenous therapy is its inability to decrease systemic pressure thereby maintaining the coronary perfusion pressure and the myocardial perfusion. In this case report, we discuss the use of NO in a routine thoracic surgery patient suffering with severe left ventricular dysfunction and a potential candidate for a very high cardiac risk.

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Aims and Objectives: We assessed the clinical impact of goal-directed coagulation management based on rotational thromboelastometry (ROTEM) in patients undergoing emergent cardiovascular surgical procedures. Materials and Methods: Over a 2-year period, data from 71 patients were collected prospectively and blood samples were obtained for coagulation testing. Administration of packed red blood cells (PRBC) and hemostatic products were guided by an algorithm using ROTEM-derived information and hemoglobin level. Based on the amount of PRBC transfused, two groups were considered: High bleeders (≥5 PRBC; HB) and low bleeders (<5 PRBC; LB). Data were analyzed using Chi-square test, unpaired t-test and analysis of variance as appropriate. Results: Pre-operatively, the HB group (n = 31) was characterized by lower blood fibrinogen and decreased clot amplitude at ROTEM compared with the LB group (n = 40). Intraoperatively, larger amounts of fibrinogen, fresh frozen plasma and platelets were required to normalize the coagulation parameters in the HB group. Post-operatively, the incidence of major thromboembolic and ischemic events did not differ between the two groups (<10%) and the observed in-hospital mortality was significantly less than expected by the Physiological and Operative Severity Score for the enumeration of Mortality and Morbidity (POSSUM score, 22% vs. 35% in HB and 5% vs. 13% in LB group). Conclusions: ROTEM-derived information is helpful to detect early coagulation abnormalities and to monitor the response to hemostatic therapy. Early goal-directed management of coagulopathy may improve outcome after cardiovascular surgery.

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