Performance and Failure of Right Ventricle to Pulmonary Artery Conduit in Congenital Heart Disease.

Surgical implantation of a right ventricle to pulmonary artery (RV-PA) conduit is an important component of congenital heart disease (CHD) surgery, but with limited durability, leading to re-intervention. The present single-center, retrospective, cohort study reports the results of surgically implanted RV-PA conduits in a consecutive series of children and adults with CHD. Patients with CHD referred for RV-PA conduit surgical implantation (from October 1997 to January 2022) were included. The primary outcome was conduit failure, defined as a peak gradient above 64 mm Hg, severe regurgitation, or the need for conduit-related interventions. Longitudinal echocardiographic studies were available for mixed-effects linear regression analysis. A total of 252 patients were initially included; 149 patients were eligible for follow-up data collection. After a median follow-up time of 49 months, the primary study end point occurred in 44 (29%) patients. A multivariable Cox regression model identified adult age (>18 years) at implantation and pulmonary homograft implantation as protective factors (hazard ratio 0.11, 95% confidence interval [CI] 0.02 to 0.47 and hazard ratio 0.34, 95% CI 0.16 to 0.74, respectively). Fever within 7 days of surgical conduit implantation was a risk factor for early (within 24 months) failure (odds ratio 4.29, 95% CI 1.41 to 13.01). Long-term use of oral anticoagulants was independently associated with slower progression of peak echocardiographic gradient across the conduits (mixed-effects linear regression p = 0.027). In patients with CHD, the rate of failure of surgically implanted RV-PA conduits is higher in children and after nonhomograft conduit implantation. Early fever after surgery is a strong risk factor for early failure. Long-term anticoagulation seems to exert a protective effect.

Impact of preoperative left pulmonary artery stenting on the Fontan procedure: a retrospective multicentre study.

OBJECTIVES: Left pulmonary artery (LPA) or bifurcation stenoses at Fontan palliation can be very challenging to treat and may also require cardioplegia and aortic transection. Moreover, the low pressure of Fontan circulation and the bulkiness of the aorta increase the risk of a patch angioplasty collapse. Pre-Fontan LPA stenting of stenotic LPAs overcomes those drawbacks therefore the present study aimed to evaluate its advantageous impact on Fontan surgery. METHODS: A multicentre retrospective analysis was performed on 304 consecutive Fontan patients. The study population was divided into 2 groups (LPA stented, n = 62 vs not stented, n = 242); pre-and postoperative data were compared. RESULTS: LPA-stented patients had a higher prevalence of systemic right ventricle (P = 0.01), hypoplastic left heart syndrome (P = 0.042), complex neonatal palliations (Norwood/Damus-Kaye-Stansel) and surgical LPA patch repair at Glenn (P < 0.001). No differences were found in cross-clamp rates, early (P = 0.29) and late survival (94.6% vs 98.4, P = 0.2) or complications (P = 0.14). Complex palliations on ascending aorta/aortic arch (P = 0.013) and surgical LPA repair at Glenn (P < 0.001) proved to be risk factors for LPA stenting before Fontan at multivariable analysis. CONCLUSIONS: The LPA-stented group showed similar outcomes in terms of survival and complications rate compared to patients without LPA stenosis; however, they significantly differ in their higher preoperative risk profile and in their more complex anatomy. Complex neonatal palliations involving ascending aorta or aortic arch may increase the risk of pulmonary branches stenosis requiring stenting; therefore, preoperative stenting of LPA stenoses could help to reduce the surgical risk of complex Fontan procedure by avoiding the need for cross-clamp or complex mediastinal dissections to perform a high-risk surgical repair.

The concept of cone creation to treat isolated tricuspid valve dysplasia and the case of a double-orifice tricuspid valve.

Objectives: Isolated tricuspid valve dysplasia is a rare disease characterized by a wide spectrum of possible anomalies. We describe the use of the Cone concept to treat a patient with a double-orifice tricuspid valve with massive regurgitation and severe deficit of coaptation. Methods: Three adult patients with congenital non-Ebstein tricuspid valve anomaly characterized by severe coaptation deficiency underwent tricuspid valve repair applying the Cone technique. In particular, we describe the case of a symptomatic 21-year-old woman with a double-orifice tricuspid valve, with massive regurgitation and severe right ventricular dilatation. The tricuspid valve was transformed from a double-orifice valve into a single-orifice valve. The most superior orifice was opened, and the tissue surrounding the orifice was used to extend the leaflet of the inferior orifice. A Cone was created, and a ring annuloplasty was used to stabilize the result. Results: The patient was discharged home after 7 days with trivial residual tricuspid regurgitation and no significant antegrade gradient. The final coaptation height was 2.8 cm. The cardiothoracic ratio decreased from 0.77 to 0.59 after 2 months, and symptoms promptly improved. Conclusions: Over the past 2 years, we have applied the Cone creation concept to patients with a severely dysplastic tricuspid valve with excellent early results. One patient had a double-orifice tricuspid valve, and a Cone repair concept was adopted anyway. One orifice was sacrificed, and surrounding tissue was used to augment the leaflets of the other orifice. A Cone was created to improve central coaptation with a good initial result.

Bentall Endocarditis by C. Lusitaniae After COVID-19: The Finger Covers The Moon.

We report a case of endocarditis months after a Bentall procedure. This was caused by Candida Lusitaniae, in an immunocompetent patient with a recent SARS-CoV-2 infection. The patient underwent a new Bentall procedure. SARS-CoV-2 has been associated with co-infection by Candida species since the beginning of the pandemic, nevertheless, Candida Lusitaniae remains a very uncommon causative agent of prosthetic endocarditis. We suggest a possible role of the SARS-CoV-2, which may have delayed the diagnosis of endocarditis and the appropriate therapy.

Staged approach for correction of anomalous venous return to the azygos vein.

Partial anomalous pulmonary venous return into the azygous vein is a rare pathological finding. We describe the case of a 28-year-old girl who had a successful staged approach to treat this rare congenital heart disease. To avoid potential connection of a systemic venous return to the left atrium, the proximal part of the azygous vein was occluded with a percutaneous approach, then the azygous vein flow was redirected into the left atrium with a surgical procedure.

Coronary Artery Aneurysms in Patients With Marfan Syndrome: Frequent, Progressive, and Relevant.

BACKGROUND: There are few data on the prevalence and clinical consequences of coronary artery aneurysms (CAAs) in adult patients with Marfan syndrome (MFS). METHODS: We performed a retrospective cohort study of 109 patients with pathogenic variants in the FBN1 gene. Diameters of the left main coronary artery (LMCA) and right- coronary artery (RCA) were measured by computed tomography angiography. RESULTS: The overall prevalence of CAA was 46%. The prevalence rates of CAA were 18% and 68% in patients with a native aortic root (group 1) and patients with previous aortic-root replacement (group 2), respectively. Previous aortic dissection or aortic intervention, longer time from aortic-root replacement, higher systemic score, significant mitral valve involvement, and diffuse aortic disease were correlated with CAA. During a mean follow-up of 8.5 ± 7.6 years, 4 patients developed pseudoaneurysms of the coronary anastomoses, requiring surgery. CONCLUSIONS: CAAs are common in adult patients with MFS and are associated with a more severe aortic phenotype and a longer follow-up after aortic-root replacement. Our study demonstrates that coronary artery size should be regularly followed, mostly after aortic-root replacement and in patients with severe aortic phenotypes. Large multicentre studies are warranted to elucidate the most appropriate surveillance plan.

Myocardial oxygen consumption during histidine-tryptophan-ketoglutarate cardioplegia in young human hearts.

OBJECTIVES: Energy demand and supply need to be balanced to preserve myocardial function during paediatric cardiac surgery. After a latent aerobic period, cardiac cells try to maintain energy production by anaerobic metabolism and by extracting oxygen from the given cardioplegic solution. Myocardial oxygen consumption (MVO2) changes gradually during the administration of cardioplegia. METHODS: MVO2 was measured during cardioplegic perfusion in patients younger than 6 months of age (group N: neonates; group I: infants), with a body weight less than 10 kg. Histidine-tryptophan-ketoglutarate crystalloid solution was used for myocardial protection and was administered during a 5-min interval. To measure pO2 values during cardioplegic arrest, a sample of the cardioplegic fluid was taken from the inflow line before infusion. Three fluid samples were taken from the coronary venous effluent 1, 3 and 5 min after the onset of cardioplegia administration. MVO2 was calculated using the Fick principle. RESULTS: The mean age of group N was 0.2 ± 0.09 versus 4.5 ± 1.1 months in group I. The mean weight was 3.1 ± 0.2 versus 5.7 ± 1.6 kg, respectively. MVO2 decreased similarly in both groups (min 1: 0.16 ± 0.07 vs 0.36 ± 0.1 ml/min; min 3: 0.08 ± 0.04 vs 0.17 ± 0.09 ml/min; min 5: 0.05 ± 0.04 vs 0.07 ± 0.05 ml/min). CONCLUSIONS: We studied MVO2 alterations after aortic cross-clamping and during delivery of cardioplegia in neonates and infants undergoing cardiac surgery. Extended cardioplegic perfusion significantly reduces energy turnover in hearts because the balance procedures are both volume- and above all time-dependent. A reduction in MVO2 indicates the necessity of a prolonged cardioplegic perfusion time to achieve optimized myocardial protection.

Aortopulmonary Collateral Artery from the Proximal Ascending Aorta: A Rare Anatomical Finding.

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries is a rare congenital heart lesion in which pulmonary blood supply may arise from different segments of the aorta. We report an unusual case of a newborn with a major collateral artery originating from the proximal ascending aorta. Successful reparative surgery was undertaken.

[Anomalous origin of the right coronary artery from the pulmonary artery or coronary artery fistula: when the diagnosis is uncertain]. / Quando la diagnosi può essere incerta: origine anomala dell'arteria coronaria destra dall'arteria polmonare o fistola coronarica?

The anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital coronary anomaly with a reported incidence of approximately 0.002%. Usually, the diagnosis is made by echocardiography leaving computed tomography or angiography only to diagnostic completion in doubtful cases or for interventional procedures.Herein we report a doubtful case of a patient with a diagnosis of coronary fistula between the right coronary and the pulmonary artery that proved to be an anomalous origin of the coronary artery from the pulmonary artery. The patient underwent corrective surgery with translocation of the coronary artery on the aorta.

Selective versus standard cerebro-myocardial perfusion in neonates undergoing aortic arch repair: A multi-center study.

The results of neonatal aortic arch surgery using cerebro-myocardial perfusion were analyzed. Selective cerebral and myocardial perfusion, using two separate pump rotors, was compared with standard perfusion, using a single pump rotor with an arterial line Y-connector. Between May 2008 and May 2016, 69 consecutive neonates underwent arch repair using either selective cerebro-myocardial perfusion (Group A, n = 34) or standard perfusion (Group B, n = 35). The groups were similar for age, weight, BSA, prevalence of one-stage or staged repair, and single ventricle palliation; male gender was more frequent in Group A. The duration of the cerebro-myocardial perfusion was comparable (27 ± 8 vs. 28 ± 7 min, P = 0.9), with higher flows in Group A (57 ± 27 vs. 39 ± 19 mL/kg/min, P = 0.01). Although cardioplegic arrest was more common in Group B (13/34 vs. 23/35, P = 0.03), the duration of myocardial ischemia was longer in Group A (64 ± 41 vs. 44 ± 14 min, P = 0.04). There was 1 hospital death in each group, with no permanent neurological injury in either group. Cardiac morbidity (1/34 vs. 7/35, P = 0.02) was more common in Group B, while extracardiac morbidity was similar in both the groups. During follow-up (3.2 ± 2.4 years), 5 late deaths occurred with a comparable 5-year survival rate (75 ± 17% vs. 88 ± 6%, P = 0.7) and freedom from arch reintervention (86 ± 6% vs. 84 ± 7%, P = 0.6). Risk of cardiac morbidity was greater with standard cerebro-myocardial perfusion (OR = 5.2, CI 3.3-6.8, P = 0.001) and with perfusion flows less than 50 mL/kg/min (OR 3.7, CI 1.87-5.95, P = 0.04). Cerebro-myocardial perfusion is a safe and effective strategy to protect the brain and heart in neonates undergoing arch repair. Selective techniques using higher perfusion flows may further attenuate cardiac morbidity.

Dacron Conduit for Extracardiac Total Cavopulmonary Anastomosis: A Word of Caution.

BACKGROUND: The extracardiac conduit technique is a valid option for completing total cavopulmonary anastomosis (TCPC) in patients with a single ventricle. The technique allows for beating heart surgery, optimal flow dynamics, and reduced postoperative atrial arrhythmia. Different types of conduit have been proposed. This study reported a single-centre experience with two different types of conduit. METHODS: Consecutive patients referred for TCPC at the current institution between January 2001 and September 2013 were included. Retrospective extraction of pertinent variables was accomplished through electronic patient chart review. Patients were stratified based on the type of conduit used to perform the TCPC: polytetrafluoroethylene (PTFE) conduit (Group A) and polyethylene-terephthalate (Dacron) conduit (Group B). RESULTS: The patient population included 105 patients: Group A had 80 patients, and Group B had 25 patients. The two groups were similar in major clinical and procedural variables, including conduit size and Nakata index. Eighteen patients (Group A: one [1.25%]; Group B: 17 [68%]) had conduit occlusion or severe stenosis requiring intervention after a mean 46.9±35months after the operation. The percentage of patients in Group A who were free of conduit obstruction at 3, 5, and 10 years was 100%, 100%, and 96%, respectively, whereas these figures were 68%, 52%, and 35% in Group B (log-rank <0.000). Conduit re-intervention was associated with an increased risk of overall mortality after primary intervention (p<0.004). Dacron tube was found to be an independent risk factor for mid-term stenosis or obstruction (hazard ratio, 62.9; 95% CI, 8.2-482.2; p=0.000). CONCLUSION: Dacron conduit for TCPC surgery was associated with a higher risk of obstruction and need for early re-intervention compared with PTFE conduit. Surgical or percutaneous re-interventions for conduit obstruction increased the risk of late mortality.

Primary Cardiac Leiomyoma Causing Right Ventricular Obstruction and Tricuspid Regurgitation.

We report the unique case of a primary cardiac leiomyoma originating from the right ventricle and involving the tricuspid valve in a 43-year-old woman. Echocardiography showed a giant mass causing severe pulmonary stenosis and tricuspid valve regurgitation. The patient underwent surgical excision and histologic examination revealed a primary cardiac leiomyoma. To the best of our knowledge only three cases of primary cardiac leiomyoma have so far been reported, and this is the first case of primary cardiac leiomyoma involving the tricuspid valve apparatus.

Primary malignant tumors of the heart: Outcomes of the surgical treatment.

BACKGROUND: Malignant cardiac tumors are rare and have an extremely poor prognosis even when complete resection is attempted. The aim of this study was to review the experience of primary malignant cardiac tumors in 2 Italian academic hospitals. METHODS: The hospital records were searched to identify patients with primary malignant cardiac tumors who underwent surgery between January 1979 and December 2012. Secondary cardiac tumors, whether metastatic or invasive, were excluded as were primary sarcomas of the great arteries. Fourteen patients selected from our institution's surgical series were identified. Eleven (78.6%) were men and 3 (21.4%) were women, and the mean age at surgery was 47.4 years. RESULTS: The most common histological type was angiosarcoma (28.6%). The mean survival was 28.8 ± 28 months and it was better in men than in women (30.5 ± 8.7 vs. 21.1 ± 3.2 months). Patients with a radical resection at the first surgery had a longer survival compared to patients with a partial resection (39.9 ± 23.2 vs. 24 ± 4 months). CONCLUSIONS: The treatment outcome for patients affected by primary malignant heart tumors remains poor. Aggressive surgery alone does not provide good results in terms of survival rate. A new multidisciplinary approach is mandatory to improve long-term survival.

Combined heart-liver transplantation: a single-center experience.

Combined orthotopic heart and liver transplantation (CHLT) is a lifesaving procedure for patients with end-stage heart-liver disease. We reviewed the long-term outcome of patients who have undergone CHLT at the University of Bologna, Italy. Fifteen patients with heart and liver failure were placed on the transplant list between November 1999 and March 2012. The pretransplant cardiac diagnoses were familial amyloidosis in 14 patients and chronic heart failure due to chemotherapy with liver failure due to chronic hepatitis in one patient. CHLT was performed as a single combined procedure in 14 hemodynamically stable patients; there was no peri-operative mortality. The survival rates for the CHLT recipients were 93%, 93%, and 82% at 1 month and 1 and 5 years, respectively. Freedom from graft rejection was 100%, 90%, and 36% at 1, 5, and 10 years, respectively, for the heart graft and 100%, 91%, and 86% for the liver graft. The livers of eight recipients were transplanted as a "domino" with mean overall 1-year survival of 93%. Simultaneous heart and liver transplantation is feasible and was achieved in this extremely sick cohort of patients. By adopting the domino technique, we were able to enlarge the donor cohort and include high-risk patients.

Cystic atrioventricular node tumor excision by minimally invasive surgery.

Cystic tumor in the atrioventricular (AV) node region is a rare cardiac primary tumor that can lead to sudden death. Our patient was a 43-year-old woman who presented with dyspnea and a first-degree AV block seen on electrocardiography. Echocardiography revealed a cystic mass attached to the inferior portion of the interatrial septum. After surgical excision of the mass, placement of a permanent pacemaker was required for complete heart blockage. Histopathologic examination revealed the mass to be a cystic tumor of the AV node region. To our knowledge, this is the first report of this condition diagnosed ante mortem and treated successfully with minimally invasive surgery.

Primary cardiac tumours in the paediatric population.

Primary cardiac tumours are relatively rare in the paediatric population, and they may occur with different signs and symptoms in foetal or post-natal life. The clinical manifestations of cardiac tumours in foetal life may include arrhythmias, congestive heart failure and hydrops. In post-natal life, cardiac tumours may cause cyanosis, respiratory distress, myocardial dysfunction, valvular insufficiency, arrhythmias, inflow or outflow tract obstructions and sudden death. Surgical treatment is essential when symptoms are present, while the role of medical therapy can merely be palliative. Results are various and related to the patients' and tumour characteristics. Primary benign heart tumours mainly have a good prognosis, while malignant neoplasms usually have a poor prognosis; in both cases, however, a strict follow-up is always mandatory in order to detect the recurrence of cardiac neoplasms after surgery.

Primary benign cardiac tumours: long-term results.

OBJECTIVES: Primary heart tumours are rare lesions with variegated histological types. We reviewed our 35 years experience with a significant number of primary benign cardiac tumour cases. METHODS: The patient database at University of Bologna was searched to identify patients with primary cardiac tumours between 1974 and 2009. Benign tumours were classified as myxomas and non-myxomas. Ninety-four were myxomas (mean age of 59.1 ± 15.6), and 13 were benign non-myxomas tumours (mean age of 39.7 ± 24.9; P = 0.0001). Complete resection of the masses was performed in all cases except in one. RESULTS: In-hospital mortality was 3% in the myxoma group and 8% in the non-myxoma group. The mean follow-up was 15.1 and 7.4 years for the myxoma and non-myxoma groups, respectively. The long-term survival of discharged patients was 68 ± 7% for the myxoma group and 100% for the non-myxoma group at 20 years, respectively. Recurrence of a tumour occurred only in the myxoma group (four cases) after 1, 3, 5 and 8 years, respectively. Twenty patients had an extracardiac tumour that was diagnosed before operation in 12 (11 in the myxoma group) and during the follow-up in 8 patients (only in the myxoma group). CONCLUSIONS: Primary cardiac tumours can be surgically treated with good short- and long-term results. Mortality and morbidity are mainly due to the status of preoperative patients'. An accurate follow-up is mandatory in order to detect the recurrence of a cardiac tumour such as to exclude the presence or the development of extracardiac tumours that we found frequently associated with the myxoma.