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Familial amyloidotic polyneuropathy (FAP) is an inherited disease characterized by an abnormal systemic deposition of a mutant protein called transthyretin (TTR) with elective involvement of the peripheral nervous system, but often determining cardiac, gastrointestinal, and urinary tract dysfunction. FAP commonly affects the liver and the heart until end-organs failure. Transthyretin amyloidosis is today an accepted indication for orthotopic liver transplantation (OLT). Combined heart and liver transplantation (CHLT) may be an attractive and rational treatment option when both organs are contemporary involved by this type of amyloidotic disease. Nowadays, surgical indications and techniques are far from being consolidated because only few cases of CHLT have been previously reported in literature. From November 1999 to May 2006, we performed five orthotopic combined heart and liver transplantations for FAP at our institution. Our surgical experience and clinical outcomes are herein reported.

Aortic complications are uncommon in cardiac allograft recipients. Primary acute aortic rupture is an extremely rare and dramatic event that can occur in the early phase after transplantation. In this article we describe a case of acute intraoperative rupture of the donor aorta just after aortic declamping during orthotopic cardiac transplantation procedure, successfully treated with a Bentall-De Bono operation.

Mitral valve myxomas are extremely uncommon, and their presence raises differential diagnosis with other pathologic conditions. We report on the case of a patient with an unspecific clinical presentation and an equivocal echocardiographic finding: mass prolapsing through the mitral valve into the left ventricle. The tumor was strongly attached by a wide pedicle, involving both the anterior and posterior mitral valve leaflets. Tumor excision was not feasible, and the mitral valve was replaced. Histologic analysis confirmed the myxomatous nature of the mass.

Cardiac lipomas are very rare neoplasms. We describe herein a case of giant intrapericardial extracavitary lipoma in a 67-year-old man who has been previously treated for prostate and kidney cancers. The patient underwent successful resection of the tumor through right anterolateral thoracotomy.

Papillary fibroelastoma is a benign but rare tumor of the heart, usually derived from the endocardium. Although rarely clinically symptomatic, fibroelastoma has a potential for valvular dysfunction and systemic embolization. In this article, we show the images of a previously undiagnosed mass that was surgically excised in a 73-year-old woman.

Heart transplantation is accepted worldwide as the treatment of choice for patients with end-stage heart disease who have a life expectancy of less than 1 year. Mechanical Cardiac assistance might be necessary as a bridge to transplantation and, in selected cases, to multiorgan transplantation. We report a successful case of a 15-day extracorporeal membrane oxygenator assistance as bridge to combined heart and liver transplantation in a young man with dilated cardiomyopathy and chronic hepatitis C virus infection.

We present a case of a transplanted patient with late acute myocardial rejection, complicated by cardiogenic shock. A long cardiopulmonary resuscitation was required. Only the circulatory support necessary to stabilize vital signs was used. Thirteen days of support resulted in complete myocardial and clinical recovery.

The current surgical strategy for pulmonary endarterectomy (PEA) involves the use of extracorporeal circulation and hypothermic circulatory arrest (HCA). The aim of the present study was to test the feasibility of a different strategy of extracorporeal circulation, which could prevent bronchial back bleeding and allow a bloodless operating field, avoiding the risks associated with HCA in patients undergoing pulmonary endarterectomy. Between June 2004 and September 2005, eight patients underwent PEA without HCA. We introduced a double venting of the left heart sections, utilizing two cannulas placed in the left ventricle and atrium. Both vent cannulas are connected with vacuum device to prevent back-bleeding and left heart distension from the large amount of bronchial flow. We were able to perform pulmonary endarterectomy avoiding circulatory arrest and deep hypothermia without sacrificing the effectiveness of the procedure. The initial encouraging results have convinced us to apply systematically this technique in the cases operated in our center, even though further investigations are necessary to fully examine this technique.

Aortic valve-sparing operations have provided very good clinical outcomes. However, the absence of the sinuses of Valsalva might limit valve durability. The Gelweave Valsalva prosthesis, which presents pre-fashioned neo-sinuses, has been designed in order to avoid early leaflets deterioration. We report our results in 63 patients who underwent valve-sparing operations (reimplantation technique) using the Gelweave Valsalva graft. The main indication was ascending aorta aneurysm or annuloaortic ectasia, with or without aortic insufficiency. The operation was performed also in cases of Marfan syndrome, Bicuspid Aortic Valve (BAV), and acute Type A dissection. In-hospital mortality was of 4.7%, and two thirds were acute Type A dissection patients (P=0.01). There were no late deaths. Three years freedom from grade 3-4 AI and freedom from late aortic valve replacement were 91.7+/-4.3% and 93.8+/-5.1%, respectively. Aortic valve-sparing operations show good results in patients electively operated for aortic root ectasia. Aortic cusps repair may lead to late failure. Even if the Gelweave Valsalva prosthesis is easy to implant and it also reproduces pseudosinuses, a long-term follow up is necessary to determine if this graft may reduce leaflets deterioration.

Aim: We have retrospectively evaluated our results after aortic root and ascending aorta reoperations to determine risk factors of early death and late mortality., Methods: From January 1986 to April 2002, 73 patients underwent ''reoperative'' procedures on the aortic root and the ascending aorta. The mean age was 56.1+/-13.4 years and males numbered 62 (84.9%). The most frequent indication for reoperation was degenerative aortic aneurysm (49.3%) followed by post-dissection aneurysm (11%). Aortic root replacement with composite valve graft was performed in 47 patients (64.4%) and with aortic homograft in 2 (2.7%). Nineteen patients (26%) underwent ascending aorta replacement with tubular graft, and 4 (5.5%) underwent tailoring aortoplasty of the ascending aorta., Results: The 30-day mortality rate was 16.4% (12 patients). Mortality following elective operations was 8%, and that following urgent or emergency operations was 34.8% (p=0.002). Late survival of hospital survivors at 1, 5 and 10 years was 93.8%, 77.7% and 37%, respectively. In the multivariate Cox regression analysis chronic renal failure (p=0.003) and urgent or emergency operation (p=0.018) were found to be independent predictors of late mortality., Conclusions: Reoperations on the ascending aorta can be accomplished with acceptable early mortality and satisfactory long-term, Results: More radical treatment of the aortic pathology at the initial operation may reduce the need for further reoperations. A careful follow-up is extremely important for detecting complications of the first operation or progression of the aortic pathology before an emergency operation, predictive of poorer early and late outcome, is needed.

Background: Minimally invasive cardiac surgery (MICS) is a safe and satisfactory approach used mainly in mitral valve surgery with excellent results in many centers. Cardioplegia administration can be still a problem, especially when an endoaortic clamp is used. We retrospectively analyzed our early results with histidine-triptophane-ketoglutarate (HTK) solution used for myocardial protection in MICS., Methods: Between February 2003 and February 2004, 8 patients underwent mitral valve surgery using an endo- cardiopulmonary bypass (CPB) system and HTK solution as myocardial protection. The mean patient age was 67.7 +/- 9.2 years, and the preoperative ejection fraction was normal in all patients. Three patients had valve repair and 5 had valve replacement. Mean CPB time was 129.2 +/- 19.4 minutes, and aortic cross-clamp duration was 88.5 +/- 15.4 minutes., Results: In every case HTK solution was used for only a single dose for cardioplegia at the beginning of the procedure, without any recalls. The heart restarted spontaneously at reperfusion in 6 of 8 cases (75%), and there were no significant modifications in electrocardiogram results or myocardial cytonecrosis enzymes (creatine kinase and its MB fraction) during the postoperative period., Conclusions: HTK solution is a cold crystalloid cardioplegia solution that has demonstrated its utility in MICS because it provides a safe long cardioplegic arrest time and it reduces the risk of inadequate coronary perfusion due to dislodgement of the endoaortic clamp.

Surgical treatment of the thoracic aorta combined with endovascular stent grafting may be an interesting approach to diseases of the thoracic aorta in high-risk patients. A patient with a chronic post-dissection aneurysm of the aortic arch and proximal descending aorta developed, after surgery, a perianastomotic leak of the distal suture of the graft. We successfully treated the leak by means of an endovascular stent graft procedure. These combined procedures may be useful to resolve surgical complications particularly in case of high operative risk.