Perioperative Hemostatic Management of a Newborn With Hereditary Hemophilia A Undergoing Emergent Surgery for Dextro-Transposition of the Great Arteries.

Hemophilia A is an inherited bleeding disorder characterized by a lack of plasma clotting factor VIII (FVIII). In prophylaxis or during surgery, FVIII infusions are necessary to prevent bleeding. The authors describe the perioperative challenges and application of a multidisciplinary hemostatic management approach to a Caucasian male newborn, with antenatal diagnoses of moderate hemophilia A (2 IU/dL) and dextro-transposition of the great arteries requiring arterial switch surgery within the first month of life. Because both conditions are rare, only few reports in the literature are available describing perioperative management of hemophilia in neonates and children undergoing cardiac surgery. After baseline FVIII determination and normal standard coagulation studies, iterative intravenous pharmacist-prepared plasma-derived FVIII boluses were calculated (35 IU/kg) and administered intravenously every 6 hours for 24 hours, then switched to a continuous infusion and guided by daily chromogenic clotting FVIII activity assay for targeted values between 80 and 100 IU/dL. Successful cardiac surgery, using cardiopulmonary bypass, was performed with continuous infusion of FVIII at 5 IU/kg/h. Thirteen days after surgery, the FVIII antibody screening remained negative and continuous infusion was switched in favor of a daily intravenous bolus treatment to facilitate reconciliation to the center of origin. The authors' multidisciplinary strategy, established antenatally, allowed for successful care in this highly complex and rare situation.

Long term outcome of laryngeal mobility disorder and quality of life after pediatric cardiac surgery.

INTRODUCTION: Laryngeal mobility disorder after a pediatric heart surgery is common (between 5 and 10% of cases), and has important consequences on swallowing, breathing and speaking. After reviewing the literature, the recovery rate is variable and the postoperative follow-up is often done on a short time frame. The primary objective of the study is to describe the recovery from laryngeal mobility disorder with a follow-up time of at least 5 years. The secondary objective is to describe of the quality of life of the child in terms of phonation and swallowing, and to identify potential risk factors for a lasting laryngeal mobility disorder. METHODS: We collected data (morphological characteristics and details of the procedures and medical care) on children who had undergone a heart surgery with risks of complications, between 2010 and 2015, and with a laryngeal mobility disorder detected after the surgery through nasal flexible laryngoscopy. During a follow-up consultation, carried at least 5 years after the surgery, we performed a nasal flexible laryngoscopy to assess whether or not the patient had recovered a full mobility of the larynx. Two questionnaires were also given to the patients, the pVHI and the PEDI EAT-10, to assess respectively the quality of their speech and of their swallowing function. RESULTS: The recovery rate for a laryngeal mobility disorder more than 5 years after surgery was found to be 65% (9 children out of the 14 included in the study). We identified a risk factor for the persistence of a laryngeal mobility disorder after surgery: the presence of an associated genetic syndrome, p = 0.025. Children with persistent laryngeal mobility disorder have an impaired quality of life score, using the pVHI scale, which correlates well with the flexible laryngoscopy findings, p = 0.033. CONCLUSION: Children with a lasting laryngeal mobility disorder have disabling respiratory and vocal symptoms in their daily lives. Nasal flexible laryngoscopy should therefore be systematically performed postoperatively after a surgery carrying risks. For improved patient management, early detection of these disorders by pharyngolaryngeal nasal flexible laryngoscopy in the aftermath of high-risk cardiac surgery is strongly advised, with prolonged follow-up.

Direct Reimplantation Procedure in Anomalous Aortic Origin of the Right Coronary Artery: Long-Term Single Center Outcomes.

BACKGROUND: Anomalous aortic origin of the right coronary artery is known to be a cause of sudden cardiac death in athletes. There are no specific guidelines concerning treatment strategy in the literature. The aim of this study is to describe and report our experience of direct reimplantation technique in the treatment of this anomaly. METHODS: This was a retrospective single center study of 30 patients who underwent surgery in the congenital heart disease unit of Louis Pradel Heart and Lung Hospital between January 2003 and December 2016. The mean follow-up was seven years (3 months-17 years). RESULTS: Thirty patients underwent surgery. The median age was 17 years (0.2-52 years). There were 24 males. The median weight was 58 kg (3.6-118 kg). Fourteen patients were actively engaged in sports. Twenty-six patients had exertional chest pain or syncope. The median time lapse between diagnosis and intervention was 4.5 months (0.5-179 months). Twenty-seven (90%) patients underwent reimplantation of the anomalous coronary artery without transverse aortotomy, while in 3 (10%) patients transverse aortotomy was used to facilitate reimplantation to avoid tension at the anastomosis. There was no early death; one late death occurred in the third postoperative month. At the last follow-up, all patients had returned to normal physical activity without evidence of ischemia. CONCLUSIONS: Direct reimplantation allows for a complete restoration of the coronary anatomy and enables patients to return to normal physical activity. Our study shows encouraging results using a direct reimplantation technique without aortotomy.

Health Profile of Precarious Migrants Attending the Médecins Du Monde's Health and Social Care Centres in France: a Cross-Sectional Study.

Objective: The present study aimed to compare the precarious migrants' health problems managed in Médecins du Monde's health and social care centres (CASO) with those of patients attending general practice in France. Methods: We compared the most frequent health problems managed in the 19 CASO in metropolitan France with those of a national sample of usual general practice consultations, after standardisation for age and sex. Results: Precarious migrants had fewer health problems managed per consultation than other patients (mean: 1.31 vs. 2.16), and these corresponded less frequently to chronic conditions (21.3% vs. 46.8%). The overrepresented health problems among CASO consultations were mainly headache (1.11% vs. 0.45%), viral hepatitis (1.05% vs. 0.20%), type 1 diabetes (1.01% vs. 0.50%) and teeth/gum disease (1.01% vs. 0.23%). Their underrepresented health problems were mainly lipid disorder (0.39% vs. 8.20%), depressive disorder (1.36% vs. 5.28%) and hypothyroidism (0.50% vs. 3.08%). Prevention issues were nominal in precarious migrants (0.16%). Conclusion: Both chronic somatic and mental conditions of precarious migrants are presumably underdiagnosed. Their screening should be improved in primary care.

Excision of Wilms' Tumor With Atrial Extension Under Moderate Hypothermia and Cerebral Perfusion.

BACKGROUND: Wilms' tumor is the most common pediatric renal tumor. Almost half of all cases have involvement of the inferior vena cava, which must be addressed at the time of surgical excision. Further extension into the right atrium may pose an immediate vital risk and necessitates special operative techniques that employ cardiopulmonary bypass. CASE REPORT: We report the case of a child with a left Wilms' tumor with inferior caval and right atrial involvement, which led to significant hemodynamic compromise and urgent surgery. A left nephrectomy and cavoatrial thrombectomy were performed via a sterno-laparotomy. Our strategy employed moderate hypothermic circulatory arrest at 26°C and antegrade cerebral perfusion in order to improve visualization and ensure complete thrombectomy and protection of the abdominal organs. CONCLUSION: This case emphasizes the advantages of moderate hypothermic circulatory arrest compared to deep hypothermic circulatory arrest and normothemic cardiopulmonary bypass.

Surgical repair of a pseudocoarctation with cervical aortic arch complicated by multiple aneurysms of the aorta: a case report.

Aortic pseudocoarctation is a rare congenital anomaly characterized by elongation and deformity of the aortic arch and is known to be associated with aneurysmal formation. Several studies unite to say it leads to a surgical sanction as soon as symptomatic or associated with aneurysms of the aortic arch. Our patient is a 12 years old boy, followed since birth for a little tight pseudocoarctation with a cervical aortic arch and transverse aortic arch hypoplasia. Close clinical and paraclinical monitoring including angioscans, showed the gradual enlargement of the superior mediastinum, in relation with the appearance of three aneurysms of the aortic arch. The intervention, performed by sternotomy, has consisted of the resection of the aneurysmal area and the interposition of a Dacron tube to repair the aortic arch and the reimplantation of the left subclavian artery into the left carotid artery. The postoperative course was uneventful. Management of pseudocoarctation associated with cervical aortic arch and aneurysms remains surgical. Close monitoring of patients with pseudocorctation, seems to be essential to avoid fatal complications such as aneurysmal rupture.

Total Anomalous Pulmonary Venous Connection: A 40 years' Experience Analysis.

Background Total anomalous pulmonary venous connection is a rare cardiac malformation associated with significant morbidity and mortality rates. We report a large surgical series study to evaluate mid-term and long-term results of conventional surgical techniques. Methods and Results We performed a retrospective analytic study of all patients operated on for simple total anomalous pulmonary venous connection in the University Hospital of Lyon, France, between January 1973 and June 2014. A total of 180 patients were included (43% supracardiac, 27% intracardiac, 19% infracardiac, and 11% mixed types). Mean cardiopulmonary bypass and aortic cross clamp times were respectively 66 and 39 minutes. Overall mortality was 27.1%, including 38 early deaths (21.1%) and 12 late deaths (6.1%). The percentage of early death greatly decreased over the eras, from 42.1% in the seventies to 7.4% after 2010. Besides the earlier era of intervention (p < 0.0001), significant risk factors for death in multivariate analysis were preoperative pulmonary hypertension, acidosis, and cardiopulmonary bypass time. There were 24 reoperations, including 7 for pulmonary venous obstruction; 6 died. Factors directly and independently associated with late complications were the anatomic type (mixed forms, p = 0.0023), and length of aortic cross clamp time (p = 0.01). Long-term results for survivals are excellent. We report 84.7% of asymptomatic patients with a mean follow-up of 10.8 years. Conclusions The overall prognosis of total anomalous pulmonary venous connection repair with conventional procedures has greatly improved over the years with excellent long-term results. A thorough evaluation of all preoperative characteristics is imperative to achieve the best outcome.

[About a case of a recurrent glandular cardiac myxoma in a child]. / À propos d'un cas de myxome cardiaque avec éléments glandulaires récidivant chez un enfant.

Primary cardiac tumors are extremely rare and mainly benign. The majority of these are myxomas (40%). Myxoma are generally sporadic tumors which occur most commonly in adult females between 30 and 40 years, and are seldom found in the paediatric population (5%). Seven percent are associated with igenetic diseases. We report the case of an eight-year-old boy presenting a recurrent glandular cardiac myxoma. In 2011, he presented a deterioration of the general state. An echocardiography highlighted a left atrial mass on the interatrial septum, with a pedicular insertion. On the microscope, it consisted of a proliferation of stellate cells isolated or clustered in rudimentary vessels in a myxoid stroma presenting haemorrhage changes. These cells expressed CD34 and calretinine. Glandular elements without atypia were clustered within the myxomatous proliferation. They expressed cytokeratin (CK) 7. Surgical resection was macroscopically complete. In 2014, the boy had a sudden neurological deficit during a football match. An echocardiography revealed a recurrence at the same location. The lesion was excised and addressed in several fragments. Classical myxoma was associated with glands without atypia. This last component expressed CKAE1/AE3 and CK7. Ki67 index of proliferation was low. The surgical reintervention was macroscopically complete. The final diagnosis was glandular cardiac myxoma. A genetic survey was conducted, showing the presence of Carney complex. This is the first description in the litterature of a recurrent glandular cardiac myxoma occuring in a child.

Successful Operation for Thoracoabdominal Aortic Aneurysm in a 5-Year-Old Boy With Tuberous Sclerosis.

The association of abdominal aortic aneurysm (AAA) and tuberous sclerosis (TS) is rare. A 5-year-old boy was diagnosed with a 7-cm calcified thoracoabdominal aortic aneurysm (TAAA), and the clinical evaluation revealed TS. The patient underwent an open repair with a 14-mm polyester tube graft prosthesis. The pathologic examination showed nonspecific dystrophic changes with loss of elastin fibers in the media of the aorta. The graft was patent on computed tomographic angiography performed 1 month after the operation. Because of the high risk of rupture, early elective repair is suggested.

Left posterolateral thoracotomy: an alternative approach for pulmonary valve replacement.

Pulmonary valve replacement in adults who have a repaired tetralogy of Fallot is realized through a redo median sternotomy. A dilated ascending aorta is often present and adherent to the sternum and can be injured during sternum reentry, with dramatic consequences. We report on an adult patient with a corrected tetralogy of Fallot who underwent pulmonary valve replacement, thick transannular patch excision, and left pulmonary artery enlargement. Surgery was performed through a left posterolateral thoracotomy. This surgical approach was safe and efficient and, compared with the left anterior thoracotomy approach, offered many more possibilities.

Tissue hemoglobin monitoring is unable to follow variations of arterial hemoglobin during transitions from pulsatile to constant flow in cardiac surgery.

OBJECTIVE: To test whether the variations of tissue hemoglobin concentration (∆THb) measured by the FORE-SIGHT(TM) cerebral oximeter can accurately detect changes in arterial hemoglobin concentration (∆AHb) before, during, and after cardiopulmonary bypass. DESIGN: A prospective clinical study. SETTING: Cardiac surgery operating room. PARTICIPANTS: Thirty patients scheduled for cardiac surgery. INTERVENTIONS: Tissue hemoglobin concentration (THb) was recorded continuously via 2 sensors applied on the forehead and connected to the cerebral oximeter. Arterial hemoglobin concentration (AHb) was measured in a hematology analyzer laboratory. Hemodynamic and respiratory parameters as well as epidemiologic data also were noted. Data were collected at 3 perioperative times: After induction of anesthesia, 10 minutes after cardioplegia, and at the end of the surgery. MEASUREMENTS AND MAIN RESULTS: Ninety pairs of data were collected. The coefficient of linear regression between ∆THb and ∆AHb was 0.4 (p<0.001). After exclusion of Hb variations<5%, the trending ability of THb to predict ∆AHb was 87%. However, the Bland and Altman plot graph for THb and AHb showed major limits of agreement (2.4 times the standard deviation). Central venous pressure and carbon dioxide tension were linked independently and positively with THb (p = 0.03). CONCLUSIONS: Continuous monitoring of THb cannot accurately track variations of AHb during the transition from pulsatile to continuous flow and vice versa in cardiac surgery. Local hemodynamic factors such as PaCO2 and vasodilation significantly impact THb. In this setting, THb monitoring should not be used to guide eventual blood transfusion management.

Could anterior papillary muscle partial necrosis explain early mitral valve repair failure?

Standardized techniques of mitral valve repair (MVR) have recently witnessed the introduction of a 'respect rather than resect' concept, the strategy of which involves the use of artificial chordae. MVR displays several advantages over mitral valve replacement in degenerative mitral regurgitation (MR), but the risk of reoperation for MVR failure must be taken into account. Different mechanisms could be advocated as the leading cause of MVR failure; procedure-related mechanisms are usually involved in early MVR failure, while valve-related mechanisms are common in late failure. Here, the case is reported of an early failure of MVR using artificial chordae that could be explained by an unusual procedure-related mechanism, namely anterior papillary muscle necrosis. MVR failure is a well-known complication after surgical repair of degenerative MR, but anterior papillary muscle partial necrosis might also be considered a possible mechanism of procedure-related MVR failure, especially when considering the increasing use of artificial chordae. Owing to the encouraging results obtained, mitral valve re-repair might be considered a viable solution, but must be selected after only a meticulous evaluation of the underlying mechanism of MVR failure.

Total percutaneous femoral vessels cannulation for minimally invasive mitral valve surgery.

BACKGROUND: Minimally invasive mitral valve surgery (MIMVS) has experienced several technological changes in the last two decades. Our aim was to describe one of the most recent improvements, the utilization of a total percutaneous femoral vessels cannulation technique during MIMVS. METHODS: We performed a retrospective observational analysis of this technique among 300 consecutive MIMVS patients, with particular focus on cannulation aspects of MIMVS, its success rate and potential complications. RESULTS: From October 2008 to December 2012, 300 patients (60% males) were operated on. Mean age was 62.9±16.4 years. Indications for operation included mitral valve repair (93%) and mitral valve replacement (7%). Two femoral arterial catheterizations failed and required conversion to sternotomy. The complications on the arterial side were: 5 (1.6%) cases of bleeding during the introduction of Prostar leading to a preoperative surgical hemostasis; 2 (0.6%) retroperitoneal bleeds during cardiopulmonary bypass requiring difficult surgical control but with an uneventful follow-up; 6 (2%) bleeding episodes after removal of the arterial cannula easily controlled by direct surgical revision; 1 (0.3%) arterio-venous fistula requiring a surgical correction on postoperative day 32; 1 (0.3%) patient had a transitory claudication due to a superficial femoral artery thrombosis progressively compensated by the collateral circulation. There were no postoperative bleeding complications. There were no other complications linked to the femoral cannulations or to the groin occurred during the follow-up. The percentage of uneventful arterial cannulations was 80% among the first 50 patients (N=10 out of 50) and 98.8% thereafter (N=3 out of 250). CONCLUSIONS: Total percutaneous femoral vessels cannulation technique is particularly suitable for MIMVS with a high success rate and few complications after a short learning curve. With the advent of the percutaneous approach, the traditional complications of the groin incision have completely disappeared in modern operations with no groin infection, hematoma or lymphocele.

Ability of the third-generation FloTrac/Vigileo software to track changes in cardiac output in cardiac surgery patients: a polar plot approach.

OBJECTIVE: To evaluate the ability of the third-generation (3.01) of FloTrac/Vigileo monitor (Edwards Lifesciences, Irvine, CA) to follow variations in cardiac output (∆CO) using the new polar plot approach. DESIGN: Prospective interventional study. SETTING: Single hospital university study. PARTICIPANTS: Twenty-five patients referred for cardiac surgery. INTERVENTIONS: CO was measured simultaneously by 3 to 5 bolus thermodilution (COtd measurements), using a pulmonary artery catheter and by arterial pulse contour analysis, using the FloTrac/Vigileo (COvi). Data were collected at eight time points: before incision, after sternotomy, before and after protamine sulfate infusion, at the start of sternal closure, at the end of surgery, on arrival to intensive care unit, and after a standardized volume expansion with 500 mL of hetastarch 6%. MEASUREMENTS AND MAIN RESULTS: One-hundred thirty-five pairs of CO data were collected; the mean bias of all CO measurements corrected for repeated measures was 0.2 L/min with limits of agreements of -3.3 L/min and +2.9 L/min. The percentage error was 66.5%. The polar plot analysis included 71 significant ∆CO and showed a mean polar angle of -3.4 degrees with 95% polar percentage error equivalent limits of -61 to 55; 69% of analysed data points fell within the 30-degree limits and provided a correct polar concordance rate. CONCLUSIONS: Third-generation FloTrac/Vigileo software still lacks the accuracy to reliably detect changes in cardiac output (∆CO) in cardiac surgery. Improvements to FloTrac/Vigileo CO algorithm and software still are needed in this particular setting.

Potential interest of a new absorbable collagen membrane in the prevention of adhesions in paediatric cardiac surgery: a feasibility study.

BACKGROUND: Open-heart surgery can result in adhesions, which can complicate resternotomy. AIMS: To document the occurrence of adhesions after the use of a new collagen membrane; to evaluate its tolerability; and to compare surgical parameters with control patients. METHODS: Paediatric patients who underwent cardiac surgery with the collagen membrane (Cova™ CARD; Biom'up, Saint Priest, France) were analysed retrospectively for levels of adhesion and tolerability. The times of dissection and intervention and the transfusion of packed red blood cells and haemostatic products were compared to a historic cohort who did not receive an anti-adhesion device. RESULTS: From January 2010 to December 2011, 36 patients received a collagen membrane. Nineteen re-interventions were performed, after a mean of 169 days. No grade 3 adhesions were observed and no tolerability problems were reported. During re-interventions after more than 30 days, the propensity score-adjusted durations of dissection and the total process for patients with and without a collagen membrane were 32 vs 41 minutes and 151 vs 182 minutes, respectively (not significant). The mean quantities of red blood cells and biological glue administered in the two groups were 98 vs 139 mL and 1.2 vs 0.5 mL, respectively (not significant). CONCLUSIONS: This feasibility study shows the potential use of the new membrane in paediatric patients, both in terms of prevention from severe adherence and tolerability. This is the first study of this membrane in humans. A prospective, controlled study is necessary to provide strong evidence of its efficiency.

Therapeutic management changes and mortality rates over 30 years in ventricular septal rupture complicating acute myocardial infarction.

Recent studies have shown that the decrease in ventricular septal rupture (VSR) incidence after acute myocardial infarction is related to the improvement of reperfusion strategies. Our main objective was to explore the influence of therapeutic management changes on post-infarct VSR patient outcomes in a single reference center over a period of 30 years. We analyzed therapeutic management strategies and mortality rates in 228 patients with VSR after acute myocardial infarction admitted from 1981 to 2010. Patients were classified in 3 successive decades. There were no significant differences in clinical characteristics of patients with VSR at admission among those decades. Overall, surgery was performed in 159 patients (71.9%), primary transcatheter VSR closure was attempted in 5 patients (2.2%), and 64 patients (27.6%) were managed medically. Independent predictors of in-hospital mortality were VSR surgical repair (odds ratio [OR] 0.22, 95% confidence interval [CI] 0.1 to 0.7, p = 0.008), cardiogenic shock (OR 6.06, 95% CI 2.8 to 13.1, p <0.0001), and Killip class on admission (OR 1.75, 95% CI 1.1 to 9.9, p = 0.02). We found a significant 1-year mortality reduction between the first and second decades (hazard ratio 0.48, 95% CI 0.28 to 0.80; p = 0.005), with no significant change in the last decade (p = 0.2). This change was related to a systematic referral to surgical repair and shorter delays to VSR surgery (5.2 ± 6.3 vs 1.9 ± 3.2 days from first to second decade; p = 0.012). In conclusion, surgical repair remains the only significant efficient therapy to reduce mortality in patients with VSR (p <10(-3)). In-hospital prognosis remains disappointing. This contrasts with the favorable long-term outcome of patients who survive the perioperative period and are discharged from hospital.

Synergy between stents and extracorporeal membrane oxygenation in multitrauma patients with inferior vena cava injury.

Despite the lack of evidence in the literature, we report the case of a 25-year-old man involved in a road traffic accident, who had an inferior vena cava (IVC) injury and severe lung contusion with parenchymal bleeding requiring an extracorporeal membrane oxygenation (ECMO). An emergency procedure to implant a stent graft was successful in repairing the IVC injury. Moreover, we think that ECMO, in addition to providing oxygenation, reduced bleeding by creating a negative pressure along the injured IVC. The patient was decannulated on the eighth day and discharged 31 days after the accident.

Hemodynamics and arteriovenous malformations in cavopulmonary anastomosis: the case for residual antegrade pulsatile flow.

OBJECTIVES: Continuous flow in Fontan circulation results in impairment of pulmonary artery endothelial function, increased pulmonary vascular resistance, and, potentially, late failure of Fontan circulation. Bidirectional cavopulmonary shunt is the interim procedure in palliation of patients with single-ventricle physiology, but pulmonary arteriovenous malformations occur in many patients. In a porcine chronic model of cavopulmonary shunt, we studied pulmonary hemodynamics, pulmonary arteriovenous malformation occurrence, and gas exchange capabilities. We hypothesized that residual antegrade pulsatile pulmonary flow may attenuate the deleterious effects of nonpulsatile Fontan-type circulation. METHODS: Thirty pigs underwent a sham procedure (n = 10, group I), a cavopulmonary shunt with right pulmonary artery ligation (n = 10, group II, nonpulsatile), or a cavopulmonary shunt with proximal right pulmonary artery partial ligation (n = 10, group III, micropulsatile). Three months later, in vivo hemodynamics, blood gas exchange, pulmonary arteriovenous malformation occurrence, and lung histology were assessed. RESULTS: At 3 months, group II right lungs demonstrated significantly increased pulmonary artery pressure, pulmonary vascular resistance, and evidence of pulmonary arteriovenous malformations compared with groups I and III (all P < .001). Group III lungs also showed increased pulmonary artery pressure and pulmonary vascular resistance compared with the sham group, but significantly less than group II. Group III right lungs had the best gas exchange performance, with less histologic changes compared with group II. CONCLUSIONS: We developed a viable chronic large animal model of bidirectional cavopulmonary anastomosis. Residual antegrade pulsatile flow in the setting of a cavopulmonary shunt prevents pulmonary arteriovenous malformation formation and attenuates, but does not suppress, the development of pulmonary hypertension. From a clinical standpoint, these data would support keeping a small amount of antegrade pulsatile flow during creation of a cavopulmonary shunt.

Focus on echocardiographic and Doppler analysis of coronary artery abnormal origin from the pulmonary trunk with mild myocardial dysfunction.

BACKGROUND: Late presentation of abnormal origin of coronary artery from the pulmonary artery (ACAPA) is uncommon compared with early presentation, which usually induces extended myocardial necrosis and severe heart failure. The late presentation is characterized by abundant development of intercoronary collaterals resulting in mild and rare symptoms, but nevertheless can cause sudden cardiac death. Our objective was to describe presentation, cardiovascular imaging methods for diagnosis and outcomes of patients with late presentation of ACAP. METHODS: The study is a retrospective review of a single-center database to identify all patients diagnosed with ACAPA beyond the first year of life. RESULTS: From 1976 to 2011, 10 patients were identified with ACAPA at the age of 1.1-64 years: 6 with left coronary artery from the pulmonary artery (ALCAPA) and 4 with right coronary artery from the pulmonary artery (ARCAPA). Echocardiography and Doppler imaging evidenced: (1) direct signs: the abnormal coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow and (2) indirect signs: abundant intercoronary septal collaterals with anterograde flow (ARCAPA) or retrograde flow (ALCAPA) and dilatation of the controlateral normally originated coronary artery. Nine patients underwent surgical implantation of the ACAPA into the ascending aorta. After 7.9 years mean follow-up, all were asymptomatic except one who required a second surgery. CONCLUSIONS: Noninvasive cardiovascular imaging, namely transthoracic echocardiography and Doppler specific parameters, can reach diagnosis of late presentation of ACAPA. Direct aortic implantation is a reliable and effective to establish dual coronary artery circulation and prevent risks due to myocardial ischemia.

Early to midterm results of total cavopulmonary connection in adult patients.

BACKGROUND: Total cavopulmonary connection (TCPC) has not been studied in adults. We investigated early and midterm morbidity and mortality in adults undergoing TCPC and assessed risk factors for mortality. METHODS: Between June 1994 and October 2010, 30 adults (21.3 ± 5.5 years) underwent TCPC (extracardiac conduit). Twenty-two patients who had palliated single ventricles underwent TCPC completions and 8 patients underwent TCPC conversions. Preoperative and perioperative data were reviewed retrospectively. RESULTS: Six of 9 patients with preoperative atrial flutter or fibrillation or intraatrial reentry tachycardia were treated in the catheterization room. An aortic cross-clamp was necessary in 12 patients, and 16 TCPCs were fenestrated. Mean follow-up was 51 months (range, 4-198 months). Early mortality was 10%: 2 of 8 conversions and 1 of 22 completions. There was 1 late conversion death (at 56 months postoperatively). Postoperatively, 4 patients required pacemakers and 1 patient required long-term antiarrhythmic medication, but no heart transplantations were necessary. Risk factors for early mortality were arrhythmia (p = 0.02), aortic cross-clamp (p = 0.054), and extracorporeal circulation in hypothermia (p = 0.03). Risk factors for overall mortality were conversion (p = 0.047), absence of fenestration (p = 0.036), surgery before January 2006 (p = 0.036), aortic cross-clamp (p = 0.018), extracorporeal circulation in hypothermia (p = 0.008), and arrhythmia (p = 0.005). New York Heart Association functional class had improved at the last follow-up: preoperatively, 17 patients were in class II and 12 patients were in class III versus 18 patients in class I and 9 patients in class II postoperatively (p < 0.001). At the last clinical visit, systemic ventricular function was maintained, and no late supraventricular arrhythmia was found. CONCLUSIONS: Early and midterm TCPC results for adults are encouraging for completion but are disappointing for conversion. Identified risk factors for mortality should improve patient selection for TCPC.