Coupling of central venous pressure and intracranial pressure in a 6-year-old patient with fontan circulation and intracranial hemorrhage

A sharp rise in intracranial pressure (ICP) was noted in a 6-year-old girl with Fontan circulation after surgical removal of a hematoma associated with intracranial hemorrhage. Inhalation of nitric oxide resulted in reduction of central venous pressure (CVP) and precipitous fall in ICP, which was otherwise resistant to conventional therapy. Specifically, the rate of fall of ICP (9 mm Hg) exceeded that of the CVP (3 mm Hg), indicating disproportionate CVP-ICP coupling. The present case provides not only a novel insight into cardio-cerebral interaction in Fontan physiology but also useful information regarding the treatment of Fontan patients in the setting of increased ICP.     

Plastic bronchitis: is thoracic duct ligation a real surgical option?

Plastic bronchitis is an unusual clinical scenario of unknown cause and occurs in multiple clinical settings. The disease is characterized by the development of arborizing, thick, tenacious casts of the tracheobronchial tree that results in airway obstruction. Patients with congenital heart disease who have undergone a Fontan operation are at high risk for having this problem develop. Management of this distressing situation is difficult with only palliative options being available, such as repeated bronchoscopies, inhaled heparin, tissue plasminogen activator, inhaled bronchodilators, or azithromycin. The patients with Fontan circuits have a myriad of unique complications develop, such as atrial arrhythmias, recurrent pleural effusions, chylothoraces, protein-losing enteropathy, and plastic bronchitis. High intrathoracic lymphatic pressures with nondemonstrable lympho-bronchial fistulas were believed to be the cause for the development of these recurrent bronchial casts in plastic bronchitis. Faced with recurrent plastic bronchitis resistant to medical management in 2 Fontan patients with normal Fontan pressures on cardiac catheterization, we decided to explore a surgical solution by performing a thoracic duct ligation. This resulted in complete resolution of the formation of casts in both patients, who were discharged home and remain asymptomatic on continued follow-up. Thoracic duct ligation provides a surgical cure for plastic bronchitis by decreasing intrathoracic lymphatic pressure and flow.     

Catheter takedown in the management of the acutely failing Fontan circulation

The acutely failing Fontan circulation requires urgent management to decompress the systemic venous pressures and augment cardiac output. This may involve the use of extracorporeal membrane oxygenation support to preserve life. Catheter-based effective Fontan takedown is an alternative to surgery in these patients, who are acutely unstable, and offers the potential for an adjustable fenestration.     

Staged hybrid left pulmonary artery rehabilitation in post-fontan left pulmonary artery hypoplasia

Left pulmonary artery hypoplasia in the setting of Fontan circulation predisposes to pulmonary artery discontinuity. We describe a novel approach to correct post-Fontan left pulmonary artery discontinuity by a strategy to produce isolated left pulmonary artery growth, followed by a catheter-based reincorporation of the left pulmonary artery into the Fontan circuit.     

Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan

A 9.5-year-old girl after Fontan procedure for hypoplastic left heart syndrome had recurrent protein-losing enteropathy (PLE) develop 2 months after partial catheter closure of the Fontan fenestration. Despite satisfactory hemodynamic measurements under general anesthesia, we postulated that she suffered from increased pulmonary vascular reactivity and commenced her on Sildenafil treatment. After 6 weeks of oral Sildenafil treatment, her serum albumin and the fecal alpha-1-antitrypsin levels normalized, and her exercise tolerance had increased. There was also an improvement of the mesenteric arterial flow patterns on Doppler studies. Sildenafil should be considered in the treatment of PLE after the Fontan procedure.     

Functional total anomalous pulmonary venous drainage by left atrium-to-superior vena cava shunt

Total anomalous pulmonary venous connection is occasionally associated with a functional single ventricle and is thought to be a risk for the staged Fontan approach. We report a patient with a very rare presentation of mitral atresia and intact atrial septum with physiologically total anomalous pulmonary venous drainage by a connecting vein between the left atrium and the superior vena cava. A surgical atrioseptectomy as a functional repair of total anomalous pulmonary venous connection and pulmonary artery banding was successfully performed as the first palliation for Fontan completion.     

Long-term survival of salvage cardiac transplantation for infective endocarditis

Cardiac transplantation has been rarely performed in patients with infective endocarditis. A 31-year-old man developed aortic endocarditis due to Brucella melitensis. He presented with fever and developed acute myocardial infarct, severe aortic regurgitation, and heart failure. Aortic valve replacement did not improve cardiac function; hence, an emergent cardiac transplantation was carried out. Eighteen years later, he is doing well and living an active and productive life. Only 6 patients have received a cardiac transplant as part of the treatment of active infective endocarditis. This patient shows how cardiac transplantation may be successfully used as salvage therapy for patients with infective endocarditis who are not candidates for valve replacement or have severe and irreversible myocardial damage.     

The efficacy of sildenafil citrate following radiation therapy for prostate cancer: temporal considerations

PURPOSE: Erectile dysfunction is a recognized complication of radiation therapy for prostate cancer. Sildenafil citrate is a well-known management strategy for erectile dysfunction that has been found to be efficacious across a wide spectrum of comorbidities, including post-radiation erectile dysfunction. We defined the efficacy of sildenafil citrate in patients with erectile dysfunction following radiation therapy for prostate cancer and assessed the impact of the interval after radiation on the success of this therapy. MATERIALS AND METHODS: Baseline and followup data on 110 patients presenting with erectile dysfunction secondary to radiation for prostate cancer was obtained. A total of 68 patients underwent 3-dimensional conformal external beam irradiation (CRT), while 42 underwent brachytherapy (BT) without androgen deprivation. All patients were considered to have erectile dysfunction after radiotherapy, as assessed by the International Index of Erectile Function (IIEF), and they were prescribed sildenafil citrate. Mean time +/- SD between the completion of radiation therapy and the initiation of sildenafil was 8 +/- 4 months. The response to sildenafil was assessed using the IIEF questionnaire. Within and between group comparisons were done for 3 time points, that is less than 12, 13 to 24 and 25 to 36 months following the completion of radiation therapy. RESULTS: The respective response rates in men who underwent BT/CRT at the 3 time points of less than 12, 13 to 24 and 25 to 36 months was 76%/68%, 54%/46% and 44%/38%, respectively. Mean IIEF erectile function domain scores for these 3 time points after BT/CRT was 26/23, 22/19 and 17/15, respectively. The percent of patients who achieved normalization of the IIEF erectile function domain at the 3 time points in the BT/CRT groups was 60%/50%, 48%/42% and 26%/19%, respectively. CONCLUSIONS: Sildenafil citrate improves erectile function in men in whom erectile dysfunction develops following radiation therapy for prostate cancer. There is a clear time dependence for the response to this therapy with a stepwise decrease in all end points examined serially in a 3-year period.     

Combined heart and liver transplantation in a patient with situs ambiguous

We report the case of 21-year-old man with Fontan circulation failure and end-stage liver cirrhosis, along with situs ambiguous. Combined simultaneous heart and liver transplantation was successfully performed with technical modifications, including rotated implantation and bilateral venous reconstruction.     

Extracardiac Fontan conversion, cryoablation, and pacemaker placement for patients with a failed Fontan

OBJECTIVE: We review our experience with Fontan conversion and cryoablation in patients with an atriopulmonary Fontan in low cardiac output from arrhythmia or venous obstruction, including two patients with protein losing enteropathy. METHODS: Fifteen patients (mean age 25.0 +/- 8.4 years) underwent extracardiac Fontan conversion, cryoablation, and pacemaker placement between November 1999 and December 2004. Twelve patients were in NYHA class III and three were in NYHA class IV. Twelve had clinically important intraatrial reentry tachycardia refractory to medical therapy. RESULTS: Follow-up was between 2 and 62 months (mean 38.4 +/- 17.7). One death occurred at seven days after surgery due to sepsis and multisystem organ failure. The second death occurred at five days from myocardial depression following surgery. One patient with PLE preoperatively died to malnutrition and sepsis on POD number 52. The second patient with protein losing enteropathy had improved NYHA classification, cessation of albumin transfusions, and a normal stool alpha antitrypsin level (down from 4.1 mg/g preoperatively). All surviving patients improved NYHA classification to class I or II. Sustained arrhythmias could not be induced in any patient. One patient had recurrence of intraatrial reentrant tachycardia eleven months postoperatively that required electrical cardioversion and is currently well controlled on one medication. The other patients are not on any antiarrhythmic medical therapy. CONCLUSION: Extracardiac Fontan, cryoablation, and pacemaker placement reduced atrial arrhythmias and improved NYHA classification. In selected patients, this operation offers improvement in clinical outcome and is an alternative to transplantation. Protein losing enteropathy may not be a contraindication to performing Fontan conversion with cryoablation.     

A Perspective on the Surgical Management of Congestive Heart Failure

Surgical treatment of patients with congestive heart failure (CHF) has steadily advanced from rescue procedures such as aneurysmectomy, rupture repair, ventricular assist devices (VADs), and transplantation to procedures that can prevent or delay the progression of cardiac dysfunction and failure. The latter include operations such as coronary artery bypass grafting (CABG) and mitral valve repair for patients with ischemic cardiomyopathy (ICMP) and mitral annular dilatation, ventricular restoration and remodeling, and cardiac resynchronization therapy. As the number of heart transplants reported worldwide continues to decline over the past decade (by over 30%), newer surgical therapies have emerged. A need arises for clinical registries such as the NIH-sponsored LVAD registry and registries for biventricular pacing and AICD implantation, for total artificial heart implants, and for mitral valve repair in patients with ICMP. Prospective trials comparing sole ventricular restoration therapy (SVR) to SVR with concomitant CABG/MVR, coronary sinus versus epicardial LV pacing for ventricular resynchronization therapy, trials comparing LVAD as destination therapy to AICD implants, mitral valve repair versus chordal-sparing valve replacement for ischemic and valvular cardiomyopathy, and off-pump versus on-pump CABG for patients with ICMP are urgently needed. Future research should also be directed toward drugs targeting “B-cell mediated” humeral vascular rejection—the Achilles heel of cardiac transplantation, xenotransplantation, permanently implantable VADs, gene therapy, and myocardial cell regeneration therapy.     

Use of Ventricular Assist Device in Univentricular Physiology: The Role of Lumped Parameter Models

Failing single‐ventricle (SV) patients might benefit from ventricular assist devices (VADs) as a bridge to heart transplantation. Considering the complex physiopathology of SV patients and the lack of established experience, the aim of this work was to realize and test a lumped parameter model of the cardiovascular system, able to simulate SV hemodynamics and VAD implantation effects. Data of 30 SV patients (10 Norwood, 10 Glenn, and 10 Fontan) were retrospectively collected and used to simulate patients' baseline. Then, the effects of VAD implantation were simulated. Additionally, both the effects of ventricular assistance and cavopulmonary assistance were simulated in different pathologic conditions on Fontan patients, including systolic dysfunction, diastolic dysfunction, and pulmonary vascular resistance increment. The model can reproduce patients' baseline well. Simulation results suggest that the implantation of VAD: (i) increases the cardiac output (CO) in all the three palliation conditions (Norwood 77.2%, Glenn 38.6%, and Fontan 17.2%); (ii) decreases the SV external work (SVEW) (Norwood 55%, Glenn 35.6%, and Fontan 41%); (iii) increases the mean pulmonary arterial pressure (Pap) (Norwood 39.7%, Glenn 12.1%, and Fontan 3%). In Fontan circulation, with systolic dysfunction, the left VAD (LVAD) increases CO (35%), while the right VAD (RVAD) determines a decrement of inferior vena cava pressure (Pvci) (39%) with 34% increment of CO. With diastolic dysfunction, the LVAD increases CO (42%) and the RVAD decreases the Pvci. With pulmonary vascular resistance increment, the RVAD allows the highest CO (50%) increment with the highest decrement of Pvci (53%). The single ventricular external work (SVEW) increases (decreases) increasing the VAD speed in cavopulmonary (ventricular) assistance. Numeric models could be helpful in this challenging and innovative field to support patients and VAD selection to optimize the clinical outcome and personalize the therapy.     

Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation.

OBJECTIVES: Hemodynamic abnormalities and refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We review our experience with conversion to total cavopulmonary artery connections and arrhythmia surgery. METHODS: Between 1994 and 2001, 40 patients underwent Fontan conversion and arrhythmia surgery. Significant hemodynamic lesions such as aortic aneurysm (n = 1), atrioventricular valve insufficiency (n = 8), and pulmonary arterioplasty (n = 9) were repaired concomitantly. Thirty-four patients were in New York Heart Association class III or IV. Mean age at the original Fontan operation was 7.5 +/- 6.5 years and mean age at Fontan conversion was 18.7 +/- 9.0 years. Arrhythmia surgery has evolved from isthmus cryoablation in 10 patients to right-sided maze in 16 patients for atrial reentry tachycardia. The maze-Cox III operation was used for 14 patients with atrial fibrillation. Atrial (n = 33) and dual-chamber (n = 5) pacemakers were placed. RESULTS: There has been no early mortality. Chest tubes were removed on postoperative day 9.0 +/- 6.0. Hospital stay was 11.8 +/- 6.6 days. Three patients required cardiac transplantation at 8 days, 9 months, and 33 months postoperatively. There was 1 death 2 years postoperatively from acute myocardial infarction. For the entire series, arrhythmia recurrence is 12.5%, with only 10% of patients receiving long-term antiarrhythmic medications; these patients were among the first 8 patients in our series. Most patients are in New York Heart Association class I or II. Bruce protocol in 12 patients showed increased tolerance (P <.05). CONCLUSIONS: Fontan conversion to total cavopulmonary connection with concomitant arrhythmia surgery is excellent therapy for patients whose Fontan repair has failed. Fontan conversion is safe, improves New York Heart Association class, improves exercise tolerance, and has a low incidence of recurrent arrhythmias.     

Pulmonary valve replacement for pacing electrodes related bacterial endocarditis

Infective endocarditis of pulmonary valve requiring surgery is rare. We report a case of 80-year-old woman with a permanent pacemaker implanted 14 years ago. She had signs of infection resistant to antibiotic therapy, secondary cachexia, and dyspnea. Echocardiography examination revealed an infectious mass on pacing electrodes in the right atrium, right ventricular dysfunction, and pulmonary hypertension. Removal of the infected pacing system was indicated. During the operation, destruction of the pulmonary valve due to bacterial endocarditis was diagnosed. Replacement with a stented porcine valve was performed. The patient was discharged on postoperative day 20. Seven months after the operation, the patient is in a good condition.     

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience

BACKGROUND: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS: Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.     

Protocols associated with no mortality in 100 consecutive Fontan procedures.

OBJECTIVES: Results of Fontan's procedure have improved considerably, but perioperative mortality still occurs, attributed to ventricular dysfunction, stroke, arrhythmia, thromboembolism, and multi-organ dysfunction. Our protocols of operative and intensive care unit management address these potential issues, and have been associated with zero mortality, even with many high-risk candidates. METHODS: From 1996 to 2006, all Fontan patients were managed as follows: operative strategy based on aortic and single atrial cannulation, cooling on full-flow bypass, and hypothermic circulatory arrest to create the Fontan pathway. No direct caval cannulation. Use of central venous lines was completely avoided. Fresh whole blood was used for pump prime and for volume restoration. Inotropic and vasodilator therapy was continued for at least 48 h. Aspirin was used exclusively as anti-thrombotic therapy. Postoperative pleural drainage was accomplished with small pigtail catheters. The usual Fontan pathway was by lateral atrial tunnel (84), with extra-cardiac conduit when dictated by anatomy (16). RESULTS: One hundred Fontan operations were performed with no mortality. All patients were extubated by postoperative day 1. Hospital stay was 10+/-5 days. Complications were: bleeding (1), reintubation (1), emergent fenestration closure (1), pericardial effusion (4), and seizures (1). Risk factors included Fontan connection to one lung (3), diminutive pulmonary arteries (PAs) and unifocalized major aortopulmonary collateral arteries (MAPCAs) (1), discontinuous PAs (3), right ventricle dependent coronaries (3), neonatal pulmonary venous obstruction (3), Trisomy 21 (1), preoperative pacemaker dependence (2), and heterotaxy (10). No candidate was excluded. CONCLUSIONS: While many surgeons try to avoid bypass or aortic clamping when performing Fontan operations, the strategies we have employed facilitate safe accomplishment of Fontan's operation in diverse anatomic groups with multiple risk factors, with avoidance of operative mortality in 100 consecutive cases.     

Utility of strain-echocardiography in current clinical practice

Myocardial strain measurement with two-dimensional speckle-tracking echocardiography (2D-STE) is of paramount importance in the early detection of subclinical left ventricular (LV) systolic dysfunction and the prediction of patient outcomes in various types of heart disease, especially when assessed with global longitudinal strain (GLS). The routine application of myocardial strain parameters requires the definition of normal values and an understanding of their reliabilities. One of the most important potential limitations to widespread clinical application of this technique is inter-vendor differences in normal strain values. Recent clinical reports indicate that the smallest differences were noted in GLS measurements among three orthogonal directions. Because the contribution of circumferential fibers to LV myocardial thickening is greater than that of longitudinal fibers, patients who have a reduced LV ejection fraction can have impaired global circumferential strain (GCS), which reflects more advanced intrinsic myocardial systolic dysfunction and is thus closely related to a poor prognosis. Since STE-derived strain analysis allows us to define the timing of the regional myocardial peak systolic deformation, it permits the assessment of LV mechanical dyssynchrony. The severity of LV mechanical dyssynchrony in the short axis plane, i.e., radial and/or circumferential strain imaging, is favorable for predicting the clinical response to cardiac resynchronization therapy. GLS in the right ventricular (RV) four-chamber view has recently been used as a surrogate for global RV function because longitudinal shortening is the major contributor to overall RV performance. Finally, 2D-STE can be used to quantify and characterize RV mechanical dyssynchrony in various diseases including acute pulmonary thromboembolism and chronic pulmonary hypertension.     

Heart transplantation in patients with previous Fontan operations

OBJECTIVE: The clinical features and outcomes of patients undergoing heart transplantation after a failed Fontan operation are still debated. The aim of this study was to retrospectively evaluate our experience in 14 patients undergoing heart transplantation after previous Fontan-type operations. METHODS: From 1990 to 2002, 14 patients underwent heart transplantation in our institution after a previous Fontan procedure. The mean age at the time of the Fontan operation and at transplantation was 7.3 +/- 2.8 and 17.2 +/- 6.3 years, respectively. The indication for transplantation was protein-losing enteropathy in 7 patients, arrhythmia with ventricular dysfunction in 5 patients, and heart failure in 2 patients. All patients received basic immunosuppressive therapy with cyclosporine (INN: ciclosporin) and azathioprine without induction therapy or maintenance steroids. RESULTS: Two hospital deaths occurred: one patient died on the fifth postoperative day of graft failure, and the second died on the 17th postoperative day after an acute neurologic event. Two patients died later, one 23 months after transplantation of acute rejection and the other after 90 months of chronic rejection and endocarditis. One patient underwent successful reintervention 2 years after heart transplantation for pulmonary vein obstruction. The 10 surviving patients are in New York Heart Association class I, with a mean follow-up of 64.5 +/- 42 months. One of them was delivered of a healthy baby 5 years after transplantation. Patients with protein-losing enteropathy reached a normal protein level within a mean of 10 months (range, 6-18 months) after transplantation. Four patients required a temporary administration (3-6 months) of oral steroid therapy for recurrent rejection episodes. Currently, 7 patients are taking cyclosporine, and 3 are taking cyclosporine and azathioprine. The actuarial survival at 1, 5, and 10 years was 86% +/- 9%, 77% +/- 12%, and 62% +/- 17%, respectively. CONCLUSION: Heart transplantation is a good option for patients with a failing Fontan operation. We documented the reversibility of protein-losing enteropathy in all patients. No mortality caused by surgical complications was observed.     

Papillary muscle approximation for functional ischemic mitral regurgitation

In patients with ischemic left ventricular dysfunction and functional mitral regurgitation, surgical treatment of mitral insufficiency remains a challenging issue. Several procedures have been described to restore a near to natural alignment between the mitral annulus and the laterally displaced papillary muscles. We report a new approach to relocate the displaced papillary muscles toward the mitral annulus and to reduce tethering in 8 patients, providing satisfactory initial results. Echocardiography showed mild or no mitral regurgitation at the follow-up (mean, 11.4 +/- 3.6 months; range = 7 to 14 months). This procedure is believed to be technically easy and beneficial in terms of mitral repair.     

RVAD implantation in a Fontan patient with protein-losing enteropathy as a bridge to transplant: Prêtre modification

Fontan patients with protein-losing enteropathy (PLE) represent poor candidates for cardiac transplantation due to end-organ injury and severely impaired clinical condition. Ventricular assist device (VAD) therapy has evolved as a promising bridge to transplant strategy improving quality of life and survival on the waiting list. However, VAD therapy for the Fontan circulation remains challenging. For Fontan patients with preserved ventricular function implantation of a right ventricular assist device (RVAD) has been described by Prêtre et al as bridge to transplant. We present the second case of RVAD support in a Fontan patient with PLE.