Double valve replacement in a patient with Maroteaux - Lamy syndrome as an ultimate team challenge.

BACKGROUND: The Maroteaux-Lamy syndrome (Mucopolysaccharidosis type VI) is a rare, inherited metabolic disease that results in progressive tissue accumulation of dermatan-sulfated glycosaminoglycans and inflammatory consequences that almost always affects the heart valves. From the anesthesia point of view, managing the airway and ventilation might be a serious challenge due to specific features of the syndrome. Additionally, it is more than probable that the surgical team will perform a non-straightforward procedure. CASE PRESENTATION: A 42-year-old male with Maroteaux-Lamy syndrome was referred to our department with shortness of breath, due to severe aortic stenosis, and at least moderate mitral valve regurgitation. The patient was initially scheduled for aortic valve replacement. After multiple attempts with video assisted laryngoscopy, the endotracheal intubation was achieved with the aid of fiberoptic bronchoscopy, while the ventilation succeeded only with laryngeal mask. The somatic features of the syndrome that made the anesthesia induction extremely difficult, also affected the surgical procedure. Suboptimal exposure of the mitral valve, patch enlargement of the aortic root to host the bigger possible prosthesis, and the hard decision to replace the mitral valve even with a marginal indication were the intraoperative challenges for the surgical team. Finally, the patient underwent a successful double valve replacement with aortic root enlargement and 18 months postoperatively remains improved. CONCLUSION: Patients with Maroteaux-Lamy syndrome represent a challenge for both anesthesiologists and cardiac surgeons. The whole team should be well prepared to deal with difficulties in airway management, ventilation and surgical valve exposure. The cardiac surgeon should be ready to offer additional procedures and even replace "prematurely" a moderately diseased valve in order to avoid a dangerous reoperation. The limited knowledge on the natural history of the Maroteaux-Lamy syndrome valvulopathy and the difficulties in anesthesia induction support this approach.

Surgical resection of a cardiac lipoma of the right ventricle.

Cardiac lipomas are rare, benign, encapsulated tumors which predominantly appear outside of the heart and less frequently inside the cardiac chambers. We describe a case of a right ventricular cardiac lipoma in a 72-year-old female patient who presented with palpitations. Surgical considerations, diagnostic modalities, and up-to-date literature are also presented and discussed.

Aortic Arch. The Final Frontier in Cardiac Surgery.

Aortic arch pathologies such as acute aortic dissection and aneurysmal disease represent surgical challenges. Various emerging techniques and surgical prostheses have expanded the therapeutic armamentarium over the last years with one principal objective; to simplify the operation and reduce the surgical time. Besides the classic elephant trunk which has been regarded as an evolutionary leap in the treatment of extensive thoracic disease, other novel surgical approaches such as the frozen elephant trunk, the thoracic endovascular aortic repair (TEVAR) and the hybrid open branched stent grafts have been introduced. This brief review aims to evaluate the surgical alternatives used in the management of complex aortic arch and proximal descending aorta pathologies with particular consideration given to the contemporary approaches which endorse single stage operation.

Late in-hospital Rupture of a Chronic Post-traumatic Pseudoaneurysm.

Chronic posttraumatic pseudoaneurysms of the thoracic aorta are rare clinical entities. Herein, we report a case of an in-hospital cervical rupture of a chronic posttraumatic false aneurysm of the aortic isthmus in a 48-year-old man who had been involved in a traffic accident 20 years earlier.

Eighteen years of clinical experience with a modification of the Bentall button technique for total root replacement.

BACKGROUND: We retrospectively reviewed our experience with the modified Bentall procedure and evaluated the short- and long-term results over a period of 18 years. METHODS: Between 1999 and 2017, 89 patients with a mean age of 57.3±13.9 years underwent the modified Bentall operation with a slight modification for the correction of aortic root disease. RESULTS: The operative mortality was 1.1% while the overall early mortality rate, defined as death within 30 days of initial hospitalization, was 2.2% (2/89). Logistic regression analysis revealed that increased Euroscore and aortic cross-clamp times were associated with greater likelihood for complications. The overall survival rates for the 89 patients (including deaths occurred at the initial hospitalization) were 93.0% (SE =3.0%) at 6 months, 93.0% (SE =3.0%) at 1 year, 89% (SE =5.0%) at 5 years and 73.0% (SE =5.0%) at 10, 15 and 18 years. Multiple Cox regression analysis for survival identified that increased aortic cross-clamp time, increased age, having a concomitant cardiac procedure and increased NYHA Class were associated with greater hazard. Left ventricular remodeling was assessed by means of echocardiography preoperatively and 1, 3, 6 and 12 months postoperatively. CONCLUSIONS: According to our experience, the Bentall procedure is a safe procedure, provides optimal long-term survival and can still be regarded as the gold standard procedure for aortic root replacement.

Distal embolization of a transcatheter valve in a valve complex: a bail-out surgical approach.

Transcatheter aortic valve replacement has emerged as an alternative to surgical aortic valve replacement for high-risk and inoperable patients. Although transcatheter aortic valve replacement avoids the use of extracorporeal circulation and sternotomy, it is nonetheless associated with inherent complications. We aim to present an embolized valve-in-valve complex in the ascending aorta, which required emergency surgery with deep hypothermic circulatory arrest and proximal aortic cannulation.

Intrapericardial bronchogenic cyst found incidentally during open heart surgery.

Bronchogenic cysts are embryological remnants occurring as developmental abnormalities of the primary foregut. The most common locations of these cysts are the mediastinum, lung parenchyma, and inferior pulmonary ligament. An intrapericardial location is an extremely rare finding. We describe the case of a 76-year-old man with aortic valve stenosis and coronary artery disease, in whom an intrapericardial bronchogenic cyst was found incidentally during the open heart procedure.

Prognostic factors of atrial fibrillation following elective coronary artery bypass grafting: the impact of quantified intraoperative myocardial ischemia.

BACKGROUND: Atrial fibrillation (AF) occurs in 28-33% of the patients undergoing coronary artery revascularization (CABG). This study focuses on both pre- and peri-operative factors that may affect the occurrence of AF. The aim is to identify those patients at higher risk to develop AF after CABG. PATIENTS AND METHODS: Two patient cohorts undergoing CABG were retrospectively studied. The first group (group A) consisted of 157 patients presenting AF after elective CABG. The second group (group B) consisted of 191 patients without AF postoperatively. RESULTS: Preoperative factors presenting significant correlation with the incidence of post-operative AF included: 1) age > 65 years (p = 0.029), 2) history of AF (p = 0.022), 3) chronic obstructive pulmonary disease (p = 0.008), 4) left ventricular dysfunction with ejection fraction < 40% (p = 0.015) and 5) proximal lesion of the right coronary artery (p = 0.023). The intraoperative factors that appeared to have significant correlation with the occurrence of postoperative AF were: 1) CPB-time > 120 minutes (p = 0.011), 2) myocardial ischemia index < 0.27 ml.m2/Kg.min (p = 0.011), 3) total positive fluid-balance during ICU-stay (p < 0.001), 4) FiO2/PO2 > 0, 4 after extubation and during the ICU-stay (p = 0.021), 5) inotropic support with doses 15-30 µg/Kg/min (p = 0.016), 6) long ICU-stay recovery for any reason (p < 0.001) and perioperative myocardial infarction (p < 0.001). CONCLUSIONS: Our results suggest that the incidence of post-CABG atrial fibrillation can be predicted by specific preoperative and intraoperative measures. The intraoperative myocardial ischemia can be sufficiently quantified by the myocardial ischemia index. For those patients at risk we would suggest an early postoperative precautionary anti-arrhythmic treatment.

Carotid shunt provides cerebral protection during emergency coronary artery bypass grafting in a patient with bilateral high grade carotid stenosis: a case report.

BACKGROUND: Management of patients with co-existent coronary and carotid disease is a controversial and challenging issue. The risk for stroke after coronary artery bypass grafting (CABG) in patients with hemodynamically significant carotid stenosis is up to 30%. In these patients a common practice is to proceed first with the restoration of cerebral perfusion and then perform the coronary revascularization. The rationale is that this strategy will reduce perioperative neurological morbidity and mortality. However, what happens when the carotid procedure is acutely complicated by cardiac instability which necessitates the interruption of the carotid procedure? CASE REPORT: We describe a case of a patient with unstable angina and high grade asymptomatic bilateral carotid stenosis who underwent emergency combined CABG and carotid endarterectomy (CEA). Due to hemodynamic instability, ST-T changes, hypotension and bradycardia, upon completion of endarterectomy we placed a carotid shunt and the patient was put on cardiopulmonary bypass through median sternotomy. After triple CABG (duration of 90 minutes) we concluded the interrupted CEA procedure with primary closure of the carotid arteriotomy with the shunt in place. The postoperative course was uneventful and the patient was discharged after a week. In extreme cases with bilateral severe carotid stenosis and coronary artery disease where the carotid procedure should be interrupted, we suggest the use of carotid shunt which can provide adequate cerebral perfusion giving time to cardiac surgeon to perform the life saving cardiac procedure first.

Transcriptional regulation of TIMPs in ascending aorta aneurysms.

The events that result in the establishment and progression of aortic aneurysms are complex and multifactorial. However, degradation of the extracellular matrix (ECM) of aortic tunica media appears to be a consistent histopathological and biochemical feature. An increased local expression of matrix metalloproteinases (MMPs) as well as an imbalance between MMP expression and the expression of their natural tissue inhibitors (TIMPs) have been demonstrated in dilated aortic wall. We hypothesized that a distinct MMP and TIMP expression pattern underlies the development of ascending aorta dilation. To test our hypothesis, expression levels of 10 MMPs and 4 TIMPs were assessed by real-time PCR in dilated and normal aortic tissue derived from patients that underwent elective surgical repair of ascending aorta aneurysm (AAA) and coronary artery by-pass grafting, respectively. We found no statistically significant up- or down-regulation of any individual MMP. Surprisingly, the tissue inhibitor of metalloproteinases (TIMP)-3 was significantly more expressed in dilated aortic tissue compared to control tissue, thereby reflecting an effort to counteract MMP activity. Finally, when we evaluated the MMP and TIMP co-expression pattern in normal and dilated aortic tissue, we observed that in aortic aneurysms activation of the MMP system was characterised by the co-expression of more than one proteinase and the down-regulation of TIMP-1 and -2. The latter observation is the key regulatory point that leads to ECM degradation and, subsequently, to AAA formation.

Stented within a stentless aortic valve. A simple surgical solution for the replacement of a stentless aortic bioprosthesis.

Stentless aortic bioprostheses are designed to offer better hemodynamics, less mechanical stress to the leaflets and as a result less degeneration. Although encouraging results are reported, little evidence has been published regarding reoperations of stentless valves. We are reporting a case of a structural valve dysfunction of an O'Brien-Angell stentless prosthesis, which could not be extracted during reoperation without damaging the aortic root. We are presenting a simple, quick and effective surgical solution, the surgical 'valve within a valve' technique for the avoidance of a redo complex root procedure.

Two stage hybrid approach for complex aortic coarctation repair.

BACKGROUND: Management of an adult patient with aortic coarctation and an associated cardiac pathology poses a great surgical challenge since there are no standard guidelines for the therapy of such complex pathology. Debate exists not only on which lesion should be corrected first, but also upon the type and timing of the procedure. Surgery can be one- or two-staged. Both of these strategies are accomplice with elevate morbidity and mortality. CASE REPORT: In the face of such an extended surgical approach, balloon dilatation seems preferable for treatment of severe aortic coarctation.We present an adult male patient with aortic coarctation combined with ascending aorta aneurysm and concomitant aortic valve regurgitation. The aortic coarctation was corrected first, using percutaneous balloon dilatation; and in a second stage the aortic regurgitation and ascending aorta aneurysm was treated by Bentall procedure. The patients' postoperative period was uneventful. Three years after the operation he continues to do well.

Asymptomatic pseudo-aneurysm of the aortic arch in a patient with aberrant right subclavian artery. A complication of Kommerell's diverticulum?

Kommerell's diverticulum is an aortic arch deformity associated with an aberrant subclavian artery. Symptoms related to compression of adjacent structures, dilatation of the aortic diverticulum or accelerated atherosclerosis leading to increased risks of dissection and rupture represent the indications for surgical treatment. Several surgical strategies have been used for the management of this congenital abnormality. We present the case of a 56-year-old male with a pseudo-aneurysm of a left aortic arch adjacent to a Kommerell's diverticulum at the orifice of a left subclavian artery. The patient also presented an aberrant right subclavian artery originating from the posterior wall of the ecstatic take-off of the left subclavian artery. Our surgical strategy was limited to the resection of the aneurysm without any manipulation of the aortic diverticulum and aberrant right subclavian artery, as the wall of both aorta adjacent to the saccular aneurysm and left subclavian artery was normal, the Kommerell's diverticulum was small and the patient was asymptomatic.

Long-term thyroid hormone administration reshapes left ventricular chamber and improves cardiac function after myocardial infarction in rats.

Thyroid hormone (TH) is critical for tissue differentiation at early stages of development, induces physiological hypertrophy and regulates the expression of important contractile proteins such as myosin heavy chain (MHC) isoform and calcium cycling proteins. Furthermore, TH seems to control the response to stress by regulating important cardioprotective molecules such as heat shock proteins (HSPs). Thus, the present study investigated whether TH administration immediately after acute myocardial infarction can favourably remodel the post-infarcted myocardium. Acute myocardial infarction was induced in rats by coronary artery ligation (AMI, n=10), while SHAM-operated animals served as controls (SHAM, n = 8). TH was administered for 13 weeks (AMI-THYR, n = 9). Cardiac contractile function and left ventricular (LV) chamber remodelling was assessed by serial echocardiography and in Langendorff heart preparations. AMI significantly reduced LV ejection fraction (EF%); 30.0 (s.e.m, 2.3) Vs. 73.8 (1.8) in SHAM, P < 0.05. In addition, +dp/dt and -dp/dt (in mmHg/s) were 4,051 (343) and 2,333 (118) respectively for SHAM Vs. 2,102 (290) and 1,368 (181) for AMI, P < 0.05. With TH treatment, EF% was increased to 49.5 (2.7) in AMI-THYR, P < 0.05, while +dp/dt and -dp/dt (in mmHg/s) were 3,708 (231) and 2,035 (95) for AMI-THYR, P < 0.05 Vs. AMI. A marked elevation of the expression of beta-MHC and a reduced ratio of SERCA/Phospholamban were found in viable myocardium of AMI hearts, which was prevented by TH. Furthermore, heat shock protein 70 myocardial content was decreased in AMI hearts and was significantly increased after TH treatment. An ellipsoidal reshaping of LV chamber was observed with TH; cardiac sphericity index, (ratio of long/short axis, SI), was 1.98 (0.03) for SHAM, 1.52 (0.05) for AMI and 1.72(0.02) for AMI-THYR, P < 0.05. In conclusion, long-term TH administration immediately after AMI results in sustained improvement of cardiac haemodynamics.

Thyroid hormone attenuates cardiac remodeling and improves hemodynamics early after acute myocardial infarction in rats.

OBJECTIVE: Cardiac remodeling of viable myocardium occurs after acute myocardial infarction (AMI) and further contributes to cardiac dysfunction. The present study explored whether thyroid hormone (TH) administered shortly after AMI in rats can attenuate cardiac remodeling and improve cardiac function. TH regulates important structural and regulatory proteins in the myocardium including myosin isoform expression and calcium cycling proteins. METHODS: AMI was induced in Wistar male rats by ligating left coronary artery (AMI, n=10), while sham-operated rats were used as controls (SHAM, n=10). Animals with acute myocardial infarction were also treated with 0.05% thyroid powder in food (AMI-THYR, n=10). Within 2 weeks, cardiac function was impaired as assessed by echocardiography and under isometric conditions in Langendorff preparations. RESULTS: Ejection fraction (EF%) was 71.5 (SEM, 2.7) in SHAM versus 30.0 (2.0) in AMI, P<0.05. +dp/dt was 3886 (566) in SHAM versus 2266 (206) in AMI hearts, P<0.05 and -dp/dt was 1860 (46) in SHAM versus 1633 (120) in AMI hearts, P=ns. Such changes were associated with alterations in myosin isoform expression in the non-infarcted area; AMI hearts expressed 34% alpha-MHC and 66% beta-MHC versus 52% alpha-MHC and 48% beta-MHC in SHAM, P<0.05, while the expression of SERCA and phospholamban (PLB) remained unchanged. Furthermore, a mismatch of left ventricular size and cardiac mass (2*Posterior Wall thickness/LVIDd was decreased) was observed. After TH treatment, AMI-THYR hearts expressed 71% alpha-MHC and 29% beta-MHC, P<0.05 versus SHAM and AMI and the ratio of SERCA/PLB was increased by 2.0-fold, P<0.05 versus SHAM and AMI. These changes corresponded to a marked improvement in cardiac function; EF% was raised to 45.8 (1.7), P<0.05 versus AMI while +dp/dt and -dp/dt were 3800 (435) and 2600 (200), respectively, in AMI-THYR hearts, P<0.05 versus AMI. The ratio of 2*Posterior Wall thickness/LVIDd was normalized. CONCLUSIONS: Thyroid hormone administration early after infarction attenuates cardiac remodeling and significantly improves myocardial performance.