Mutational Signature Analysis Reveals NTHL1 Deficiency to Cause a Multi-tumor Phenotype.

Biallelic germline mutations affecting NTHL1 predispose carriers to adenomatous polyposis and colorectal cancer, but the complete phenotype is unknown. We describe 29 individuals carrying biallelic germline NTHL1 mutations from 17 families, of which 26 developed one (n = 10) or multiple (n = 16) malignancies in 14 different tissues. An unexpected high breast cancer incidence was observed in female carriers (60%). Mutational signature analysis of 14 tumors from 7 organs revealed that NTHL1 deficiency underlies the main mutational process in all but one of the tumors (93%). These results reveal NTHL1 as a multi-tumor predisposition gene with a high lifetime risk for extracolonic cancers and a typical mutational signature observed across tumor types, which can assist in the recognition of this syndrome.

A comparison of sufentanil vs. remifentanil in fast-track cardiac surgery patients.

We retrospectively compared patients receiving remifentanil with patients receiving sufentanil undergoing fast-track cardiac surgery. After 1:1 propensity score matching there were 609 patients in each group. The sufentanil group had a significantly longer mean (SD) ventilation time compared with the remifentanil group; 122 (59) vs. 80 (44) min, p < 0.001 and longer mean (SD) length of stay in the recovery area; 277 (77) vs. 263 (78) min, p = 0.002. The sufentanil group had a lower mean (SD) visual analogue pain score than the remifentanil group; 1.5 (1.2) vs. 2.4 (1.5), p < 0.001 and consumed less mean (SD) piritramide (an opioid analgesic used in our hospital); 2.6 (4.7) vs. 18.9 (7.3) mg, p < 0.001. The results of our study show that although remifentanil was more effective in reducing time to tracheal extubation and length of stay in the recovery area, there was an increased requirement for postoperative analgesia when remifentanil was used.

Near-infrared spectroscopy monitoring of the collateral network prior to, during, and after thoracoabdominal aortic repair: a pilot study.

OBJECTIVE: The aim of this study was to evaluate the feasibility of non-invasive monitoring of the paraspinous collateral network (CN) oxygenation prior to, during, and after thoracoabdominal aortic repair in a clinical series. METHODS: Near-infrared spectroscopy optodes were positioned bilaterally-over the thoracic and lumbar paraspinous vasculature-to transcutaneously monitor muscle oxygenation of the CN in 20 patients (age: 66 ± 10 years; men = 11) between September 2010 and April 2012; 15 had open thoracoabdominal aortic repair (Crawford II and III), three had thoracic endovascular aortic repair (TEVAR; Crawford I), and two had a hybrid repair (Crawford II). CN oxygenation was continuously recorded until 48 hours postoperatively. RESULTS: Hospital mortality was 5% (n = 1), 15% suffered ischemic spinal cord injury (SCI). Mean thoracic CN oxygenation saturation was 75.5 ± 8% prior to anesthesia (=baseline) without significant variations throughout the procedure (during non-pulsatile cooling on cardiopulmonary bypass and with aortic cross-clamping; range = 70.6-79.5%). Lumbar CN oxygenation (LbS) dropped significantly after proximal aortic cross-clamping to a minimum after 11.7 ± 4 minutes (74 ± 13% of baseline), but fully recovered after restoration of pulsatile flow to 98.5% of baseline. During TEVAR, stent-graft deployment did not significantly affect LbS. Three patients developed relevant SCI (paraplegia n = 1/paraparesis n = 2). In these patients LbS reduction after aortic cross-clamping was significantly lower compared with patients who did not experience SCI (p = .041). CONCLUSIONS: Non-invasive monitoring of CN oxygenation prior to, during, and after thoracoabdominal aortic repair is feasible. Lumbar CN oxygenation levels directly respond to compromise of aortic blood circulation.

Increased risk of late aortic events after isolated aortic valve replacement in patients with bicuspid aortic valve insufficiency versus stenosis.

AIM: Bicuspid aortic valve (BAV) is a very heterogeneous disorder and risk of aortic events in BAV may be influenced by phenotype of the disease. Correlation has been proposed between aortic dilatation patterns and functional status of the BAV (i.e., stenosis versus insufficiency). The aim of our study was to evaluate the risk of late aortic events after isolated aortic valve replacement (AVR) in patients with BAV stenosis versus insufficiency. METHODS: Review of our institutional BAV database identified 442 consecutive BAV patients (mean age 55±12 years, 76% men), who underwent isolated AVR from 1995 through 2005. A subgroup of 376 (85%) patients presented with an isolated/predominant BAV stenosis (Group I), whereas 66 (15%) patients had an isolated BAV insufficiency (Group II). Follow-up information (a total of 3864 patient-years) was 100% complete. Mean follow-up period was 9.4±3.9 years. Adverse aortic events were defined as the need for proximal aortic surgery or the occurrence of aortic dissection/rupture, or sudden death during follow-up. RESULTS: Actuarial survival rates in Group I and Group II were 86±2% vs. 94±3% at 10 years, and 76±3% vs. 85±6% at 15 years, respectively (P=0.2). Proximal aortic surgery was performed in 6 (1.5%) patients in Group I vs. 2 (3%) patients in Group II. Freedom from proximal aortic re-interventions was 95±3% in Group I vs. 90±8% in Group II at 15 years after AVR (P=0.4). Aortic dissection/rupture occurred in 2 patients in Group II. Freedom from late adverse aortic events was 93±3% in Group I vs. 78±9% in Group II at 15 years postoperatively (P=0.02). CONCLUSION: BAV patients with isolated valve insufficiency are at increased risk of late aortic events, as compared with BAV stenosis patients at 15 years after AVR.

Ursodeoxycholic acid counteracts celecoxib in reduction of duodenal polyps in patients with familial adenomatous polyposis: a multicentre, randomized controlled trial.

BACKGROUND: Due to prophylactic colectomy, mortality in patients with familial adenomatous polyposis (FAP) has changed, with duodenal cancer currently being the main cause of death. Although celecoxib reduces duodenal polyp density in patients with FAP, its long-term use may increase the risk of cardiovascular events and alternatives need to be explored. Preclinical studies suggest that the combination of celecoxib with ursodeoxycholic acid (UDCA) is a potentially effective strategy. We performed a randomized, double-blind, placebo-controlled trial to investigate the effect of celecoxib and UDCA co-treatment on duodenal adenomatosis in patients with FAP. METHODS: Patients with FAP received celecoxib (400 mg twice daily) and UDCA (1000-2000 mg daily, ~20-30 mg/kg/day, n=19) or celecoxib and placebo (n=18) orally for 6 months. Primary outcome was drug efficacy, assessed by comparing duodenal polyp density at pre- and post-intervention by blinded review of endoscopic recordings. As secondary outcomes, cell proliferation, apoptosis, and COX-2 levels in normal duodenal mucosa were assessed by immunohistochemistry or real-time quantitative polymerase chain reaction. RESULTS: In intention-to-treat analysis, deceased polyp density was observed after celecoxib/placebo treatment (p=0.029), whereas increased polyp density was observed after celecoxib/UDCA treatment (p=0.014). The difference in change in duodenal polyp density was statistically significant between the groups (p=0.011). No changes in secondary outcomes were observed. Thirty patients (81%) reported one or more adverse events, 16 patients (84%, Common Toxicity Criteria for Adverse Events version 3.0 (CTCAE) grade 1-3) treated with celecoxib/UDCA and 14 patients (78%, CTCAE grade 1-2) treated with celecoxib/placebo. Nine patients (24%) discontinued intervention prematurely, 5 patients (26%) treated with celecoxib/UDCA and 4 patients (22%) treated with celecoxib/placebo. CONCLUSIONS: Celecoxib reduces duodenal polyp density in patients with FAP, and unexpectedly, high dose UDCA co-treatment counteracts this effect. The benefit of long term use of celecoxib for duodenal cancer prevention needs to be weighed against the (risk of) adverse events. TRIAL REGISTRATION: http://ClinicalTrials.gov, identifier NCT00808743.

Experience with anatomically orientated devices for transapical aortic valve implantation.

Transcatheter aortic valve implantation (TAVI) is a new technology, which is rapidly growing to a routine procedure amenable for patients with symptomatic aortic valve stenosis and higher than average risk for conventional aortic valve surgery. The crucial disadvantage of TAVI remains the not well foreseeable risk of more than trivial degree of paravalvular leakage and a high rate of atrioventricular block and consecutive pacemaker implantation. In addition, current implantation techniques do not allow controlling the rotation of first-generation devices that might be beneficial regarding optimal physiological valve performance, optimal coronary flow and avoidance of placement of covered commissures in front of the coronary ostia. These shortcomings had pushed the development of second-generation self-expandable nitinol-based devices for subcoronary implantation that aim a reduction of paravalvular leak and AV-block by anatomical orientated positioning into the aortic root. This review focuses on the description of three different TAVI concepts, which are presently under early clinical evaluation, or have recently received commercial approval, using the transapical approach.

Chromosome 20p11 gains are associated with liver-specific metastasis in patients with colorectal cancer.

OBJECTIVE: Metastatic colorectal cancer (CRC) cells have a selective preference for certain target organs that cannot be explained by circulatory patterns alone. This study aimed to identify clinicopathological features and chromosomal aberrations of primary tumours associated with organ-specific CRC metastasis. DESIGN: Clinicopathological features were investigated in patients with CRC who had exclusively hepatic (n=182) versus exclusively extrahepatic (n=139) metastases. A total of 139 primary tumours of patients with hepatic (n=85) and extrahepatic metastases (n=54) were screened for chromosomal aberrations by microarray-based comparative genomic hybridisation, and the findings were validated in an independent set of 80 primary tumours. A publicly available database was used to correlate chromosomal aberrations with gene expression. Protein expression was evaluated by immunohistochemistry on tissue microarrays. RESULTS: Patients with hepatic metastases were significantly more often male (71% vs 53% p=0.002), more often had abnormal lactate dehydrogenase activity (37% vs 14% p<0.0001), exhibited primary tumour localisation in the colon (52% vs 40% p=0.03) and had synchronous onset of metastases (70% vs 19% p<0.0001). Primary tumours of patients with hepatic metastases were more commonly T3 tumours (79% vs 63% p=0.006) and less commonly of mucinous histology (5% vs 16% p=0.02). Gain of 20p11 was more often observed in patients with hepatic metastases (p<0.05), which was confirmed in an independent dataset (p<0.05; false discovery rate <0.05). Twelve genes mapping at 20p11 were significantly overexpressed as a consequence of 20p11 copy number gain. C20orf3 showed the strongest correlation between RNA expression and DNA copy number. This was reflected in significantly higher protein expression in patients with hepatic metastases (59%; n=325) than in those with extrahepatic metastases (41%; n=256) (p=0.01). CONCLUSION: C20orf3 mapping at 20p11 is associated with hepatic-specific metastasis in patients with CRC. This gene is a candidate biomarker for liver metastases and may be of clinical value in early-stage CRC.

Acute aortic dissection type A.

BACKGROUND: Acute aortic dissection type A (AADA) is a life-threatening vascular emergency. Clinical presentation ranges from pain related to the acute event, collapse due to aortic rupture or pericardial tamponade, or manifestations of organ or limb ischaemia. The purpose of this review was to clarify important clinical issues of AADA management, with a focus on diagnostic and therapeutic challenges. METHODS: Based on a MEDLINE search the latest literature on this topic was reviewed. Results from the German Registry for Acute Aortic Dissection Type A (GERAADA) are also described. RESULTS: Currently, the perioperative mortality rate of AADA is below 20 per cent, the rate of definitive postoperative neurological impairment approaches 12 per cent and the long-term prognosis after surviving the acute phase of the disease is good. Many pathology- and therapy-associated factors influence the outcome of AADA, including prompt diagnosis with computed tomography and better cerebral protection strategies during aortic arch reconstruction. Endovascular technologies are emerging that may lead to less invasive treatment options. CONCLUSION: AADA is an emergency that can present with a wide variety of clinical scenarios. Advances in the surgical management of this complex disease are improving outcomes.

Beyond KRAS mutation status: influence of KRAS copy number status and microRNAs on clinical outcome to cetuximab in metastatic colorectal cancer patients.

BACKGROUND: KRAS mutation is a negative predictive factor for treatment with anti-epidermal growth factor receptor (EGFR) antibodies in metastatic colorectal cancer (mCRC). Novel predictive markers are required to further improve the selection of patients for this treatment. We assessed the influence of modification of KRAS by gene copy number aberration (CNA) and microRNAs (miRNAs) in correlation to clinical outcome in mCRC patients treated with cetuximab in combination with chemotherapy and bevacizumab. METHODS: Formalin-fixed paraffin-embedded primary tumour tissue was used from 34 mCRC patients in a phase III trial, who were selected based upon their good (n = 17) or poor (n = 17) progression-free survival (PFS) upon treatment with cetuximab in combination with capecitabine, oxaliplatin, and bevacizumab. Gene copy number at the KRAS locus was assessed using high resolution genome-wide array CGH and the expression levels of 17 miRNAs targeting KRAS were determined by real-time PCR. RESULTS: Copy number loss of the KRAS locus was observed in the tumour of 5 patients who were all good responders including patients with a KRAS mutation. Copy number gains in two wild-type KRAS tumours were associated with a poor PFS. In KRAS mutated tumours increased miR-200b and decreased miR-143 expression were associated with a good PFS. In wild-type KRAS patients, miRNA expression did not correlate with PFS in a multivariate model. CONCLUSIONS: Our results indicate that the assessment of KRAS CNA and miRNAs targeting KRAS might further optimize the selection of mCRC eligible for anti-EGFR therapy.

Patent foramen ovale. Correct route for implantation of a biventricular permanent pacemaker?

INTRODUCTION: Intentional or unintentional placement of a pacemaker lead into the left ventricle is an uncommon clinical entity that is associated with a high risk for systemic embolization and enormous difficulties in case of explantation. Unintentional implantation through a patent foramen ovale via the mitral valve is the usual pathway for this malposition. METHODS: We report a case where a pacemaker lead was placed intentionally into the left ventricle via a patent foramen ovale for biventricular pacing for resynchronization therapy. Later, the patient developed life-threatening pacemaker lead-associated endocarditis with sepsis. Emergency open heart surgery for lead removal was necessary in the form of a reoperation after bypass graft surgery a number of years earlier. CONCLUSION: Although it is technically feasible to implant the pacemaker lead into the left ventricle via a patent foramen ovale, we consider this option to be obsolete for use with a biventricular pacemaker, due to the multitude of risks, which can, in part, be life-threatening for the patient.

Prediction of the annuloplasty ring size in patients undergoing mitral valve repair using real-time three-dimensional transoesophageal echocardiography.

AIMS: We sought to investigate the additional value of real-time three-dimensional transoesophageal echocardiography (RT 3D TOE)-guided sizing for predicting annuloplasty ring size during mitral valve repair. METHODS AND RESULTS: In 53 patients undergoing elective mitral valve repair, an RT 3D TOE was performed pre- and post-operatively. The digitally stored loops were imported into a software for mitral valve assessment. The annuloplasty ring size was predicted by superimposing computer-aided design (CAD) models of annuloplasty rings onto Live 3D zoom loops, measurement of the intercommissural distance, or the height of the anterior mitral leaflet. The surgeon implanted the annuloplasty ring according to the usual surgical technique and was blinded to the echocardiographic measurement results. Pre-operative correlation between the selected ring size with mitral valve assessment and the actual implanted annuloplasty ring size was 0.91. The correlation for measurement of the intercommissural distance was 0.55 and for measurement of the height of the anterior mitral leaflet 0.75. The post-operative correlation with the actual implanted ring size was 0.96 for mitral valve assessment, 0.92 for intercommissural distance, and 0.79 for the anterior mitral leaflet height. CONCLUSION: Superimposition of annuloplasty ring CAD models on the Live 3D zoom loops of the mitral valve using mitral valve assessment is superior to two-dimensional measurements of the intercommissural distance or the height of the anterior mitral leaflet in predicting correct annuloplasty ring size.

Dislocation of a transapically implanted aortic valve prosthesis with a functionally bicuspid aortic valve and ascending aortic aneurysm.

In recent years, catheter-based aortic valve interventions have become established procedures for the treatment of high-risk and advanced age patients with aortic valve pathologies. One of the limitations of the widespread applicability of this procedure is the annulus size. Until recently, no prosthesis was available to treat patients with a large annulus. We report on a patient with high-grade aortic stenosis (AS) and a 27-mm annulus, who underwent transapical implantation (TAP) of an Edwards SAPIEN® 29-mm prosthesis (Edwards LifeScience, Irvine, CA, USA). Due to insufficient dilation of his heavily calcified, functionally bicuspid aortic valve leaflets during balloon aortic valvuloplasty (BAV), the TAP prosthesis did not anchor adequately. This was determined during follow-up as he developed progressive aortic insufficiency and orthopnea, and an echocardiography revealed that the valve had been displaced into the LVOT. A conventional aortic valve replacement and ascending aorta replacement were performed, at which time the TAP prosthesis was removed. The patient recovered uneventfully, and was discharged with a well-functioning aortic bioprosthetic valve and in good general condition.

KRAS mutation analysis: a comparison between primary tumours and matched liver metastases in 305 colorectal cancer patients.

BACKGROUND: KRAS mutation is a negative predictive factor for treatment with anti-epidermal growth factor receptor antibody in metastatic colorectal cancer (CRC). KRAS mutation analysis is usually performed on primary tumour tissue because metastatic tissue is often not available. However, controversial data are available on the concordance of test results between primary tumours and corresponding metastases. We assessed the concordance of KRAS mutation status in a study of 305 primary colorectal tumours and their corresponding liver metastases. METHODS: Patients with histologically confirmed CRC who underwent surgical resection of the primary tumour and biopsy or surgical resection of the corresponding liver metastasis were included. KRAS mutation analysis was performed for codons 12 and 13. RESULTS: KRAS mutation was detected in 108 out of 305 primary tumours (35.4%). In 11 cases (3.6%), we found a discordance between primary tumour and metastasis: 5 primary tumours had a KRAS mutation with a wild-type metastasis, 1 primary tumour was wild type with a KRAS mutation in the metastasis, and in 5 cases the primary tumour and the metastasis had a different KRAS mutation. CONCLUSION: We observed a high concordance of KRAS mutation status of 96.4% (95% CI 93.6-98.2%) between primary colorectal tumours and their corresponding liver metastases. In only six patients (2.0%; 95% CI 0.7-4.2%), the discordance was clinically relevant. In this largest and most homogenous study to date, we conclude that both primary tumours and liver metastases can be used for KRAS mutation analysis.

Life-threatening hemothorax resulting from lung hernia after minimally invasive mitral valve surgery.

Lung hernia following minimally invasive mitral valve surgery is an uncommen entity. We report the case of a male patient who developed a lung hernia as a sequela to limited access mitral valve surgery. Two months after discharge, the patient presented with a bulge in the region of the lateral thoracotomy related to respiration which could be provoked by a Valsalva maneuver. In the night following admission the patient had acute cardiovascular decompensation with worsening dyspnea, pallor and hypotension. The patient was quickly transferred to the ICU, where a chest X-ray revealed the presence of a large hemothorax with compression of the entire right lung. We transferred the patient to the operation room, evacuated the hemothorax and reconstructed the 15-cm long and 3-cm wide dehiscence using a GoreTex patch adapted in a special technique.

Late onset Takotsubo cardiomyopathy after mitral valve replacement.

We report a case of delayed onset Takotsubo cardiomyopathy (TC) in a 69-year-old woman, associated with minor stressors, two weeks after mitral valve replacement. After suffering several minor complications she had fully recovered and her discharge was planned. On the 14th postoperative day she had to be resuscitated due to cardiogenic shock. TC was diagnosed based on reduced ventricular function with apical ballooning and normal coronaries. Treatment with catecholamines and intra-aortic balloon pump led to full recovery. She continues to do well two years after surgery. TC should be considered as a potential cause of delayed ventricular dysfunction in postcardiac surgery patients.

Mitral valve re-replacement in a patient with osteogenesis imperfecta.

A 34-year-old patient with osteogenesis imperfecta (OI) underwent previous double-valve replacement in the aortic and mitral valve position. Bioprostheses were implanted because of an elevated risk of daily injury. Five years later the patient underwent re-replacement of the stenotic stentless mitral valve prosthesis. A right anterolateral mini-thoracotomy was used for operative access during both procedures, in order to preserve thorax stability. Patients with OI may benefit from minimally invasive valve surgery for primary procedures or reoperation.

Pacemaker-associated thrombotic occlusion of the inferior vena cava causing liver failure.

Pacemaker implantation using endocardial leads can give rise to thrombotic venous occlusion. We report the case of a 23-year-old male with transposition of the great arteries, who had previously undergone a Senning repair at the age of one year. A sick sinus syndrome required pacemaker implantation with subsequent multiple lead revisions. Following the implantation of the last lead, the patient developed complete occlusion of the inferior vena cava (IVC) with stenosis of the superior vena cava (SVC) with pacemaker leads in both lesions. Liver failure, ascites and esophageal varices developed. Thrombolytic treatment was ineffective; finally the patient was listed for liver transplantation. We explanted the lead embedding the thrombosis, together with some lead remnants. The stenosis of the SVC and the occlusion of the IVC were dilatated and stabilized with four stents. Over a follow-up period of 4 months, NYHA class improved from NYHA III to NYHA I-II, the hepatic function showed complete remission, and a liver transplantation was not necessary.

Markers for EGFR pathway activation as predictor of outcome in metastatic colorectal cancer patients treated with or without cetuximab.

BACKGROUND: Anti-EGFR monoclonal antibodies in metastatic colorectal cancer (mCRC) treatment are only effective in patients with KRAS wild type tumours. Here we assess the predictive value of other potential relevant markers involved in the epidermal growth factor receptor (EGFR) signalling pathways for response to cetuximab-based treatment. MATERIALS AND METHODS: Formalin-fixed paraffin-embedded colorectal cancer tissue of the primary tumour was obtained from 559 mCRC patients treated with chemotherapy and bevacizumab with or without cetuximab (phase III CAIRO2 study). DNA was isolated for mutation analysis of BRAF (V600E), KRAS (codon 12 and 13) and PIK3CA (exon 9 and 20). Tissue microarray's (TMA's) were constructed for the assessment of EGFR and HER2 gene copy number (GCN), and EGFR and PTEN protein expression. The results of these markers, individually or in combination, were correlated with progression-free survival (PFS) and overall survival (OS) in the subgroup of patients with a KRAS wild type tumour treated in the cetuximab-arm. KRAS wild type patients treated without cetuximab were used as a control group. RESULTS: A total of 208 tumours (39.4%) contained a KRAS mutation, 8.7% a BRAF mutation and 9.9% a PIK3CA mutation. Loss of PTEN expression and the presence EGFR protein expression were observed in 42.0% and 61.7% of the samples, respectively. An increased EGFR GCN was observed in 15.3% of the samples, and 11.5% of the evaluable samples contained an increased HER2 GCN. In KRAS wild type patients treated with cetuximab a BRAF mutation was significantly and independently associated with PFS and OS. In patients treated without cetuximab the PFS and OS were also associated with the BRAF genotype. No prognostic or predictive value was observed for any of the other markers when tested individually or in combination. CONCLUSIONS: BRAF genotype is correlated with PFS and OS in KRAS wild type mCRC patients, which is independent of cetuximab treatment. PIK3CA mutation, loss of PTEN expression, EGFR GCN and HER2 GCN have no predictive value for response to treatment with cetuximab, neither individually nor in combination with other markers.

Cerebral flow pattern monitoring by transcranial Doppler during cardiopulmonary resuscitation.

We describe the transcranial Doppler pattern during a period of cardiopulmonary resuscitation in a patient undergoing a transcatheter off-pump aortic valve implantation. Transcranial Doppler of the middle cerebral artery flow was measured as a part of a separate ongoing study. The patient developed a cardiac arrest during deployment of the valve prosthesis. The incidental presence of the transcranial Doppler allowed us to assess middle cerebral artery flow during cardiopulmonary resuscitation in real time. An initial lack of a diastolic flow pattern seen with transcranial Doppler at the beginning of cardiopulmonary resuscitation led to volume loading and optimisation of the resuscitation technique. After increasing the depth of external cardiac massage, the cerebral flow pattern improved to produce sufficient diastolic flow. The transcranial Doppler provided additional information during cardiopulmonary resuscitation, which was helpful in clinical management. The use of transcranial Doppler may be helpful for other cardiac procedures where cerebral malperfusion may occur.

Late perforation of a right ventricular pacing lead: a potentially dangerous complication.

Four days after uncomplicated implantation of a two-chamber pacemaker and a normal postoperative course, a patient was referred to our hospital with left-sided hemothorax and early hemorrhagic shock. Chest X-ray and CT scan were suspicious of a right ventricular lead perforation with additional pericardial and pleural injury. Immediate surgery was performed via a lateral thoracotomy and the perforation was repaired via direct suture. An epimyocardial ventricular lead was implanted simultaneously. The patient made an uneventful recovery.