Heart Transplantation in a Patient With a Heterotopic Percutaneous Tricuspid Valve Prosthesis: A Case Report.

The bicaval transcatheter prosthesis (TricValve) allows the treatment of cava reflux in patients with severe tricuspid regurgitation and high surgical risk. It consists of the implantation of 2 self-expanding valves in both vena cava without directly approaching the native tricuspid valve. Heart transplantation in this setting may require some modifications compared with the conventional bicaval technique. We describe the clinical case of a 69-year-old woman with a background of rheumatic mitral valve disease who required a mitral valve replacement a few decades before. Ongoing clinical deterioration with biventricular dysfunction and severe tricuspid regurgitation was treated with a percutaneous bicaval heterotopic self-expanding valve system, with no clinical benefit. The patient underwent an elective heart transplantation. For the surgical approach, venous cannulation was performed percutaneously for both the right internal jugular and right femoral vein. Due to the impossibility of extracting percutaneous caval valves, the biatrial technique was selected for heart implantation. The postoperative course was difficult, but the patient was successfully discharged home 2 months postoperatively. She remains in good clinical condition with normal heart function 1 year after the transplant. To our knowledge, this is the first report describing a heart transplant in a patient with a bicaval transcatheter prosthesis.

Heart Transplantation and Thromboembolic Risk In Patients With Percutaneous Aortic Valve Prosthesis and Left Ventricular Assist Device: A Case Report.

Transcatheter aortic valve replacements have become a good option for patients with aortic valve disease and high surgical risk. Thromboembolism is reported as a relatively common complication of Transcatheter aortic valve replacement. This risk may be increased in patients who have left ventricular assist devices, as valve motion is drastically reduced. We present the case of a 65-year-old man with a history of alcoholism and valvular cardiomyopathy (severe chronic aortic insufficiency) with severe left ventricular dysfunction. Improvement in ventricular function followed the cessation of alcohol consumption, but still in poor clinical condition. Due to his high surgical risk, it was decided to implant a percutaneous aortic valve to treat the valve deficiency. Two months later, he suffered from a first episode of embolic stroke, which he successfully recovered. Despite the treatment, his clinical status did not improve (INTERMACS 3-4), and he was referred for heart transplantation. A left ventricular assist device was implanted as a bridge to the transplant. While on the waiting list, he suffered from other 3 episodes of stroke, 2 of them requiring mechanical thrombectomy. Finally, his transplant was performed with a favorable postoperative clinical course. Heart transplantation may be feasible in patients with multiple intracardiac devices in left chambers, although we must be aware of the increased thromboembolic risk, especially when used in combination.

Timing of surgical repair of the stented aortic arch and coarctation in neonates.

We report a neonate with aortic arch hypoplasia and coarctation, in whom patency of the arterial duct could not be re-established and who was too ill to undergo primary surgical correction safely. This patient was treated in two stages: 1) angioplasty and stenting, 2) surgical correction. The safe period for surgical correction may be 2-3 weeks after angioplasty and stenting.

Mechanical ventilation during cardiopulmonary bypass in neonates improves postoperative outcome.

OBJECTIVES: Cardiopulmonary bypass generates a systemic inflammatory response. This inflammatory response is reduced if patients are ventilated during bypass, as evidenced by lower levels of postoperative circulating inflammatory mediators. However, this does not appear to make much clinical difference in adults, at least not consistently, but, to our knowledge, has never been assessed in paediatric cardiac surgery, which is the objective of this study. METHODS: This is a prospective clinical study of 12 consecutive neonates operated for the correction of either transposition of the great arteries ± ventricular septal defect or aortic arch hypoplasia ± ventricular septal defect, who were ventilated during cardiopulmonary bypass. These were compared to 11 neonates with the same malformations, who had undergone the same operations but without being ventilated during bypass (historical control group). RESULTS: One patient from the control group died on the 15th postoperative day due to sepsis and multi-organ failure. Bypass times and cross-clamp times were similar in the 2 groups. Ventilation on bypass was associated with significantly lower postoperative serum concentrations of C-reactive protein, shorter mechanical ventilation and lower vasoactive-inotropic score. Duration of stay on intensive care unit (ICU) showed a tendency to be shorter in patients who were ventilated on bypass, but this did not reach statistical significance. There were no differences between the 2 groups with respect to postoperative mixed venous oxygen saturations and serum concentrations of lactate and troponin I. CONCLUSIONS: Mechanical ventilation during cardiopulmonary bypass in neonates improves postoperative outcome.

The Predicament of Surgical Correction of Tetralogy of Fallot.

Surgery for tetralogy of Fallot has a long history, which may be described as both a success story and a failure story. It is a success story because prognosis without surgery is very poor, but surgery makes it possible for affected babies to reach adulthood and lead productive lives. It is a failure story, however, since we still cannot cure this condition; we can only palliate it as illustrated in the sobering long-term outcome of affected patients. In this review article, we aim to explore the reason for this failure. This may be summed up in terms of the nature of the obstruction to the right ventricular outflow tract, which characterizes this malformation and must be relieved. This obstruction has several possible components, but none may be eliminated without harming the ventricle. There seems to be no 'extra' muscle band in tetralogy of Fallot that may be dispensed with without undermining ventricular function; every muscle band that is there should be there, just like in the normal heart, except that these are thicker than normal and somewhat displaced in tetralogy of Fallot, thus narrowing the right ventricular outflow tract. Consequently, ventricular function deteriorates with every muscle band that is cut, just like in the normal heart. We have to harm the heart in order to repair it. Every repaired Fallot is inevitably a damaged heart. Consequently, repair of this condition cannot be curative at present; it is palliative surgery.

A New Method for Atrioventricular Valve Repair in Atrioventricular Septal Defect.

The left atrioventricular valve in atrioventricular septal defect is conventionally repaired by suturing the "cleft." This can be complicated with stretching or dehiscence of the sutured zone by naturally diverging cordal traction and stenosis by restricting leaflet mobility. This complication prompted us to develop a different approach, especially for adults and adolescents, who might have undergone surgical repair previously. The technique consists of not closing or reopening the cleft, constructing neocordae connecting the cordless edge of each bridging leaflet to the opposite papillary muscle-that is, the papillary muscle to which it has no natural connection ("crossing neocordae")-and inserting an annuloplasty ring.

Donor-specific circulating cell free DNA as a noninvasive biomarker of graft injury in heart transplantation.

BACKGROUND: Considerable effort has been exerted to develop noninvasive diagnostic biomarkers that might replace or reduce the need to perform endomyocardial biopsies. In this context, graft DNA circulating on transplant recipients has been proposed as a potential biomarker of organ rejection or cellular graft injury. METHODS: We propose a digital PCR (dPCR) method based on the amplification of ten specific InDels sufficiently sensitive to detect small amounts of specific donor circulating DNA diluted on the host cell free DNA (cfDNA). We obtained 23 informative mismatches from 30 host and donor organ biopsy pairs. RESULTS: Patients without heart-related complications showed a high increase in the specific genomic marker levels during the first 24 h after transplantation that dropped to the basal levels on days 3-4 post-surgery. In contrast, patients with complications presented a significantly lagged decay pattern from day one after transplantation. A specific donor cfDNA increase was detected in one patient two days before rejection diagnosis, diminishing the basal levels after successful immunotherapy. A cfDNA increase was also observed during graft injury due to heart damage. CONCLUSION: These results suggest that cfDNA monitoring of transplanted patients may be a useful tool to detect and probably anticipate graft rejection.

Mitral valve repair in infective endocarditis is not inferior to valve replacement: results from a Spanish nationwide prospective registry.

INTRODUCTION: Infective endocarditis (IE) still carries high morbidity and mortality and frequently requires surgery. The benefit of mitral valve repair (MVr) in the setting of IE is yet to be proven. The goal of this study was to assess the results of MVr in patients with IE after a minimum follow-up of 1 year. METHODS: This study is based on a Spanish nationwide prospective registry that included patients operated on for native mitral valve IE. The collaborating Institutions pooled their pre-, peri-, and postoperative data into the database of the GAMES group [Grupo de Apoyo al Manejo de la EndocarditiS (Group for support and management of infective endocarditis)]. RESULTS: Data from 27 hospitals were recorded and 3524 cases of active IE identified between 2008 and 2016. There were 1513 cases of mitral IE, of which 898 involved native valves. Of these, 437 patients underwent surgical treatment, and 369 completed the 1-year follow-up. The valve was repaired in 68 cases (18.4%). Preoperative groups were comparable (EuroSCORE MVr 7.7 vs MVR 8.0; p = ns). Mortality in the repair group was inferior to that in the replacement group (16.2% vs 27.2%, p = 0.058). At 1 year, mortality remained higher in the replacement group: 3.7% vs 2.9%. Relapse of the infection was slightly more frequent in the repair group (7.1% vs 3.7%; p = ns), although this did not lead to higher rates of reintervention (MVr/MVR: 2.9% vs 4.9%). CONCLUSION: MVr is an attractive option for specific patients with IE and does not seem to negatively impact on relapses.

The morphologically right and left ventricles cannot be distinguished by their coronary arterial pattern.

OBJECTIVES: The morphologically right and left ventricles are distinguished from each other based on their internal anatomical features, because their external (epicardial) surfaces do not appear to have any distinguishing mark for such ventricular identification. Nevertheless, ventricular identification based on epicardial characteristics, if these were possible, would be interesting to surgeons, because this would enable them to identify each ventricle rapidly upon opening the chest. This made us curious as to whether or not the two ventricles may be distinguished based on their epicardial coronary arterial patterns, because this is the most obvious epicardial ventricular feature. METHODS: This idea led us to formulate the following 2 hypotheses: (i) The morphologically left ventricle is always the one that receives the higher number of the marginal arteries as compared to the morphologically right ventricle. (ii) Only the morphologically left ventricle receives the diagonal arteries from the anterior and posterior interventricular arteries. These hypotheses were tested in this anatomical observational study by examination of 98 normal and 398 congenitally malformed formaldehyde-preserved hearts encompassing most malformations, including rare ones and hearts in which 1 ventricle is hypoplastic. RESULTS: These examinations show that both hypotheses are false. CONCLUSIONS: The two ventricles cannot be distinguished from each other based on the number of marginal arteries that they receive or which one receives diagonal arteries; both ventricles may receive diagonal arteries from either or both interventricular arteries.

Prevalence and prognostic significance of pulmonary artery aneurysms in adults with congenital heart disease.

BACKGROUND: Prevalence and prognostic significance of pulmonary artery (PA) dilatation in congenital heart disease (CHD) have never been studied systematically. METHODS: Chest X-rays of 1192 consecutive adults with CHD were reviewed. Major diameter of the PA was determined by imaging techniques in those with PA dilatation. A value >29 mm was considered abnormal. Data on anatomy, hemodynamics, residual lesions and outcomes were retrospectively collected. RESULTS: Overall prevalence of PA dilatation was 18%. A minority of patients (5.5%) reached 40 mm (aneurysm; PAA) and 1.8% exceeded 50 mm. The most common PAA underlying malformations were pulmonary stenosis (21%), and shunts (55%). Significantly larger diameters were observed in hypertensive shunts (40 mm; IQR 36.7-45 mm vs. 34 mm; IQR 32-36 mm) (p < 0.0001). However, the largest diameters were found in cono-truncal anomalies. There was no significant correlation between PA dimensions and systolic pulmonary pressure (r = -0.196), trans-pulmonary gradient (r = -0.203), pulmonary regurgitation (PR) (r = 0.071) or magnitude of shunt (r = 0.137) (p > 0.05 for all). Over follow-up, 1 sudden death (SD) occurred in one Eisenmenger patient. Complications included coronary (3), recurrent laryngeal nerve (1) and airway (1) compressions, progressive PR (1), and PA thrombosis (1). Coronary compression and SD were strongly associated (univariate analysis) with pulmonary hypertension (120 vs. 55 mm Hg; p = 0.002) but not with extreme PA dilatation (range: 40-65 mm). CONCLUSIONS: PA dilatation in CHD is common but only a small percentage of patients have PAA. Clinical impact on outcomes is low. Complications occurred almost exclusively in patients with pulmonary hypertension whereas PA diameter alone was not associated with adverse outcomes.

Three-dimensional printed models for surgical planning of complex congenital heart defects: an international multicentre study.

OBJECTIVES: To evaluate the impact of 3D printed models (3D models) on surgical planning in complex congenital heart disease (CHD). METHODS: A prospective case-crossover study involving 10 international centres and 40 patients with complex CHD (median age 3 years, range 1 month-34 years) was conducted. Magnetic resonance imaging and computed tomography were used to acquire and segment the 3D cardiovascular anatomy. Models were fabricated by fused deposition modelling of polyurethane filament, and dimensions were compared with medical images. Decisions after the evaluation of routine clinical images were compared with those after inspection of the 3D model and intraoperative findings. Subjective satisfaction questionnaire was provided. RESULTS: 3D models accurately replicate anatomy with a mean bias of -0.27 ± 0.73 mm. Ninety-six percent of the surgeons agree or strongly agree that 3D models provided better understanding of CHD morphology and improved surgical planning. 3D models changed the surgical decision in 19 of the 40 cases. Consideration of a 3D model refined the planned biventricular repair, achieving an improved surgical correction in 8 cases. In 4 cases initially considered for conservative management or univentricular palliation, inspection of the 3D model enabled successful biventricular repair. CONCLUSIONS: 3D models are accurate replicas of the cardiovascular anatomy and improve the understanding of complex CHD. 3D models did not change the surgical decision in most of the cases (21 of 40 cases, 52.5% cases). However, in 19 of the 40 selected complex cases, 3D model helped redefining the surgical approach.

Follow-up of a simple method for aortic valve reconstruction with fixed pericardium in children.

In 2013, we published a simple method for aortic valve reconstruction in children using fixed pericardium in this journal. This was not expected to be a durable solution but a temporizing measure in the absence of other solutions, in order to buy time for growth that would allow subsequent prosthetic valve insertion. As such, this was implemented in 3 patients with excellent immediate results, as reported in our original publication. We are now writing to provide longer term information. In 2 cases, a newborn and a 12-year-old boy, the valve calcified heavily at 4 years' follow-up. By then, the valvar annulus had grown sufficiently to accept a prosthetic valve in both cases. In these 2 cases, the material used for valve reconstruction was autologous pericardium fixed in 0.6% gluteraldehyde. In the third patient, a 3-year-old girl, the valve failed by dehiscence of the suture line 6 months following surgery. In this case, fixed bovine pericardium had been used, which is more rigid than autologous pericardium, thus possibly explaining the dehiscence. However, the pericardial patch was not calcified. Thus, the described method lived up to our expectation of buying time for growth for later insertion of a prosthetic valve in 2 of the 3 cases.

A simple surgical technique to prevent atrial reentrant tachycardia in surgery for congenital heart disease†.

OBJECTIVES: To present and test a simple surgical technique that may prevent atrial reentrant tachycardia following surgery for congenital heart disease. This arrhythmia is one of the commonest long-term complications of such a surgery. It may occur many years (even decades) after the operation. It is usually explained as a late consequence of right atriotomy, which is an inherent component of many operations for congenital heart disease. Right atriotomy results in a long scar on the right atrial myocardium. This scar, as any scar, is a barrier to electrical conduction, and macro-reentrant circuits may form around it, causing reentrant tachycardia. However, this mechanism may be counterchecked and neutralized by our proposed method, which prevents reentrant circuits around right atriotomy scars. METHODS: The proposed method is implemented after termination of cardiopulmonary bypass and tying the venous purse-strings. It consists of constructing a full-thickness suture line on the intact right atrial wall from the inferior vena cava (IVC) (a natural conduction barrier) to the atriotomy incision. This suture line is made to cross the venous cannulation sites if these are on the atrial myocardium (rather than being directly on the venae cavae). Thus, the IVC, atriotomy and cannulation sites are connected to each other in series by a full-thickness suture line on the atrial wall. If this suture line becomes a conduction barrier, it would prevent reentrant circuits around right atrial scars. This was tested in 13 adults by electroanatomical mapping. All 13 patients had previously undergone right atriotomy for atrial septal defect closure: 8 of them with the addition of the proposed preventive suture line (treatment group) and 5 without (control group). RESULTS: In all 13 cases, the atriotomy scar was identified as a barrier to electrical conduction with electrophysiological evidence of fibrosis (scarring). In the 8 patients with the proposed suture line, this had also become a scar and a complete conduction barrier. In the 5 patients without this suture line, there was free electrical conduction between the IVC and atriotomy scar. CONCLUSIONS: The proposed suture line becomes a scar and conduction barrier. Therefore, it would prevent reentrant circuits around atrial scars and their consequent arrhythmias.

Three-Dimensional Transesophageal Echocardiography in the Anatomical Assessment of Isolated Parachute Mitral Valve in an Adult Patient.

Parachute mitral valve (PMV) is a rare congenital anomaly of the mitral valve apparatus usually evidenced in infants and young children. Adult presentation is extremely rare and is generally mild in terms of mitral stenosis. A 73-year-old woman was admitted to the emergency department due to progressive dyspnea, with NYHA functional class IV symptoms on presentation. The echocardiographic examination identified a PMV with moderate mitral stenosis and a secondary smaller subvalvular mitral orifice. The report shows the usefulness of three-dimensional transesophageal echocardiography in the detection and quantification of this rare anomaly.

Surgical technique for heart transplantation: a strategy for congenital heart disease.

The standard techniques for orthotopic heart transplantation often require certain adjustments when the procedure is carried out for complex congenital heart disease. This is because of both the unusual anatomy and possible distortions caused by previous surgery. Such technical adjustments have been described in various published reports over the years. Those reports, when combined, do cover the full spectrum of the technical difficulties that may be encountered, whether the defects are in their original form or altered by surgery, such that no cardiac malformation or distortion would prohibit transplantation. However, those reports are comprehensive only when combined. None of the individual reports addresses all the possible technical challenges. Consequently, the available information is somewhat fragmented. In addition, the generic aspect of the described technical strategies is not always given the emphasis that it deserves. Indeed, occasionally a technique may be presented as a specific solution for a specific malformation, without necessarily pointing out that the same technique may be applied to other hearts with different overall pathologies but which share that specific malformation. The aim of this review article was to combine all the available published information in one article in a manner that constructs a simple but comprehensive and generic system of decision-making that may be applied to any heart in order to determine the exact technical adjustments needed for transplantation in each case. Such a strategy is possible for two reasons. First, only a few anatomical sites are technically significant, namely the points of anastomosis between the donor's organ and the recipient. The rest of the intracardiac morphology does not affect the operation and may be ignored. Second, each of those anatomical sites can present difficulties in only a few ways, and each of those few difficulties has a well-described and published solution already. Therefore, the exact technical adjustments required in each case may be worked out by the sequential assessment of the anastomotic sites alone.

A simple method of aortic valve reconstruction with fixed pericardium in children.

Aortic valve reconstruction with fixed pericardium may occasionally be very useful when treating children with aortic valve disease. This is because diseased aortic valves in children are sometimes too dysmorphic for simple repair without the addition of material, their annulus may be too small for a prosthesis, and the Ross operation may be precluded due to other congenital anomalies such as pulmonary valvar or coronary malformations. Such reconstruction is usually technically demanding and requires much precision. We describe a simple alternative method, which we have carried out in 3 patients, aged 1 week, 3 years and 12 years, respectively, with good early results.