1438

CASE REPORT ROSSANO ET AL TOTAL ARTIFICIAL HEART IN THE FONTAN

Congenital Heart Database. J Thorac Cardiovasc Surg 2008; 135:546–51. 2. Okamoto T, Nishijima E, Maruo A, et al. Congenital tracheal stenosis: the prognostic significance of associated cardiovascular anomalies and the optimal timing of surgical treatment. J Pediatr Surg 2009;44:325–8. 3. Petrucci O, O’Brien SM, Jacobs ML, et al. Risk factors for mortality and morbidity after the neonatal Blalock-Taussig shunt procedure. Ann Thorac Surg 2011;92:642–52. 4. Dobrolet NC, Nieves JA, Welch EM, et al. New approach to interstage care for palliated high-risk patients with congenital heart disease. J Thorac Cardiovasc Surg 2011;142: 855–60.

Successful Use of the Total Artificial Heart in the Failing Fontan Circulation Joseph W. Rossano, MD, David J. Goldberg, MD, Stephanie Fuller, MD, Chitra Ravishankar, MD, Lisa M. Montenegro, MD, and J. William Gaynor, MD FEATURE ARTICLES

Division of Cardiology, Department of Pediatrics, Division of Pediatric Cardiac Surgery, Department of Surgery, and Department of Anesthesia and Critical Care Medicine, The Cardiac Center, The Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania

Typical left ventricular assist devices are often ineffective for the failing Fontan circulation. We report the first successful use of a total artificial heart as a bridge to transplant in a patient who had previously undergone a Fontan operation. (Ann Thorac Surg 2014;97:1438–40) Ó 2014 by The Society of Thoracic Surgeons he Fontan operation is the final planned palliative operation for children with functional single ventricles (SVs). This circulation is usually tolerated initially, but eventually fails secondary to chronic low cardiac output and elevated venous pressure. In the failing Fontan circulation, placement of a ventricular assist device (VAD) may not address the underlying circulatory impairments. Implantation of a total artificial heart (TAH) restores a “normal biventricular circulation” to potentially reverse systemic manifestations of Fontan failure and improve patient candidacy for transplantation.

T

A 13-year-old male with pulmonary atresia and intact ventricular septum developed severe, refractory circulatory failure 10 years after an extracardiac Fontan operation. He was hospitalized at an outside institution with severely depressed left ventricular systolic function,

Accepted for publication June 17, 2013. Address correspondence to Dr Rossano, The Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104; e-mail: [email protected].

Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc

Ann Thorac Surg 2014;97:1438–40

respiratory failure, hepatic insufficiency with coagulopathy, and plastic bronchitis. On transfer to our hospital, the patient was in extremis with multiorgan failure and respiratory decompensation. Echocardiography and catheterization confirmed severely depressed ventricular function with elevated Fontan pressures. With informed consent from the family, a 70-cc Syncardia TAH (SynCardia Systems, Inc, Tucson, AZ) was implanted (Fig 1). Cardiopulmonary bypass was initiated through the femoral vessels. Because of the extracardiac conduit (ECC) Fontan pathway the remaining native right atrial (RA) cavity was too small to receive superior vena cava (SVC) and inferior vena cava (IVC) flow (Fig 2A). Therefore, in order to insert the TAH, it was necessary to construct a “neo-RA.” After aortic cross-clamping, the ECC and SVC were separated from the right pulmonary artery, which was reconstructed with a homograft patch. Because the existing ECC was too small to serve as the neo-RA, a 24-mm Gor-Tex graft (W.L. Gore and Associates, Flagstaff, AZ) was inserted as an interposition graft between the IVC and the SVC to serve as a neo-RA. The left ventricle was excised, preserving the mitral annulus and a cuff of ventricular muscle. The rudimentary right ventricle was resected. The remaining free wall of the RA was used to close the atrial communication, creating a pathway for drainage of the right pulmonary veins to the left atrium. An atrial connector for the TAH was sutured to the mitral annulus and cuff of left ventricular muscle. An ellipse of the Gore-Tex graft was excised and an atrial connector sutured to the neo-RA. The pulmonary artery bifurcation was opened and the pulmonary artery outflow graft anastomosed in an end-to side fashion. The aortic outflow graft was anastomosed in an end-to-end fashion to the aorta. The drive lines for the artificial ventricles were tunneled to the left upper quadrant. The artificial ventricles were filled with saline and attached to the atrial connectors and the outflow grafts (Fig 2B). The postoperative course was complicated by significant bleeding and external compression of the neo-RA and reconstructed IVC that required reexploration and eventual stent placement. Additional complications included rhabdomyolysis and renal failure necessitating renal replacement therapy. Therapy with inhaled tissue plasminogen activator and rigid bronchoscopy were necessary to clear the airway of the casts resulting from plastic bronchitis. Cardiac output was well preserved with the VAD rates between 120 and 160, percent systole at 50% to 55%, and a cardiac output ranging from 5.5 to 75 L/minute. Over several weeks, the patient demonstrated recovery of all end-organ function, including resolution of plastic bronchitis. The patient underwent rehabilitation therapy and was able to ambulate without assistance. On postoperative day 61, a suitable donor heart became available and the patient underwent orthotopic heart transplantation. The patient suffered a cardiac arrest on posttransplant day 5 related to severe graft dysfunction from combined acute antibody mediated and cellular rejection. The 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.06.120

Ann Thorac Surg 2014;97:1438–40

CASE REPORT ROSSANO ET AL TOTAL ARTIFICIAL HEART IN THE FONTAN

1439

patient was resuscitated with extracorporeal membrane oxygenation but suffered neurologic injury. For several weeks he was not able to speak, required a tracheostomy, and had significant weakness precluding ambulation. The patient achieved significant recovery of neurologic function after inpatient rehabilitation and is currently at home without a tracheostomy and is ambulatory.

Comment This case represents the first successful use of a TAH as a bridge to heart transplantation in the failing Fontan circulation. The Fontan operation is an end-state for many forms of congenital heart disease characterized by a

functional SV. While the Fontan operation may create a durable circulation for decades, there is a growing population of children and young adults who develop “Fontan failure” as a result of the chronic state of low cardiac output and elevated venous pressure inherent in this physiology. Manifestations of Fontan failure include ventricular dysfunction (systolic and diastolic), arrhythmias, protein losing enteropathy, and plastic bronchitis. Although the current VADs allow for successful bridge to transplantation for many patients with congenital heart disease, the limited experience in patients with “Fontan failure” has been less promising [1–3]. A VAD to support the SV may have utility in patients with heart failure related to isolated ventricular dysfunction, but will not

Fig 2. (A) Extracardiac conduit (ECC) Fontan. (B) Removal of ECC, creation of “neo-right atrium” to collect caval flow, and insertion of right and left articial ventricles for the total artifical heart.

FEATURE ARTICLES

Fig 1. (A) The preoperative and (B) postoperative chest x-rays. Note the 4 artificial valves.

1440

CASE REPORT AL-ADHAMI ET AL BI-VAD SUPPORT IN SAH-INDUCED CARDIOGENIC SHOCK

References 1. Frazier OH, Gregoric ID, Messner GN. Total circulatory support with an LVAD in an adolescent with a previous Fontan procedure. Tex Heart Inst J 2005;32:402–4. 2. Sadeghi AM, Marelli D, Talamo M, Fazio D, Laks H. Shortterm bridge to transplant using the BVS 5000 in a 22-kg child. Ann Thorac Surg 2000;70:2151–3. 3. VanderPluym CJ, Rebeyka IM, Ross DB, Buchholz H. The use of ventricular assist devices in pediatric patients with univentricular hearts. J Thorac Cardiovasc Surg 2011;141:588–90. 4. Nathan M, Baird C, Fynn-Thompson F, et al. Successful implantation of a Berlin heart biventricular assist device in a failing single ventricle. J Thorac Cardiovasc Surg 2006;131: 1407–8. aussler A, Bettex D, Genoni M. Right-sided uni5. Pretre R, H€ ventricular cardiac assistance in a failing Fontan circulation. Ann Thorac Surg 2008;86:1018–20. ˇ

FEATURE ARTICLES

suffice for those with failure of the Fontan circulation [4]. Because of the passive flow of blood through the pulmonary vascular bed, inadequate preload is often the underlying cause of diminished cardiac output in the failing Fontan circulation. A systemic VAD does not address this fundamental issue and will not necessarily reduce the systemic venous pressure. Indeed, filling and function of the VAD is likely to be limited by inadequate preload. Because of poor results with isolated systemic VADs for the failing Fontan circulation, some have constructed biventricular assist devices for use in patients in functional SV [4]. There is also a single report of a Fontan takedown and placement of a sub-pulmonary VAD [5], but this strategy is subject to significant challenges related to matching of programmed pulmonary blood flow to systemic cardiac output and has not been adopted in broader clinical practice. Providing biventricular support in this clinical setting has the advantage of reducing systemic venous pressure while augmenting cardiac output. This strategy addresses the fundamental difficulty with the failing Fontan circulation and, as in this case, may allow for the resolution of symptoms and end-organ dysfunction associated with failing Fontan physiology, thus improving suitability for transplantation. It is important to note that once stable on the TAH, our patient had a complete resolution of his plastic bronchitis prior to his transplant. By providing normal cardiac output and low systemic venous pressures, the TAH may allow for resolution of the myriad manifestations of the failing Fontan circulation, thereby facilitating rehabilitation and improving suitability for eventual transplantation. The 70-cc TAH was utilized in our patient. This device is recommended for patients with a body surface are of 1.7 m2 or greater. While many younger patients with failing Fontan circulations are too small to be eligible for this device, the Food and Drug Administration recently approved a 50-cc device suitable for smaller patients. As demonstrated in this report, these novel devices are well suited for the cohort of children and young adults with failing Fontan physiology and may become an increasing important tool in the long-term care of this unique and growing population.

Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc

Ann Thorac Surg 2014;97:1440–3

Ventricular Assist Devices as Rescue Therapy in Cardiogenic Shock After Subarachnoid Hemorrhage Ahmed Al-Adhami, MRCS, Alistair Macfie, FRCA, Calan Mathieson, FRCS, Isma Quasim, FRCA, Robyn Smith, FRCA, Stewart Craig, FRCS, Roy Gardner, FRCP, John Payne, FRCP, Mark Petrie, FRCP, and Saleem Haj-Yahia, FRCS Department of Transplantation and Mechanical Circulatory Support, Golden Jubilee National Hospital; Department of Bioengineering, University of Strathclyde; Department of Anesthesiology, Golden Jubilee National Hospital; Department of Neurosurgery, Institute of Neurological Sciences, Glasgow, United Kingdom

We review the journey to myocardial and neurologic recovery of a 42-year-old mother with severe acute cardiogenic shock and multiorgan failure after extensive subarachnoid hemorrhage, who was salvaged successfully using a CentriMag short-term biventricular assist device. (Ann Thorac Surg 2014;97:1440–3) Ó 2014 by The Society of Thoracic Surgeons

C

ardiac abnormalities occur in as many as 50% of patients with subarachnoid hemorrhage (SAH) [1]. Possible hypotheses include multivessel coronary spasm, microvascular dysfunction, and catecholamine-mediated injury [1]. The most severe form of cardiac abnormality, neurogenic stressed (or stunned) myocardium, occurs in 20-30% of patients [2]. Left ventricular (LV) dysfunction limits tolerance to hypervolemic, hypertensive, and hemodilutional (“triple-H”) therapy that is classically instituted to reverse ischemic deficits from SAH-induced cerebral vasospasm [2, 3]. Inotropic therapy and intraaortic balloon bump (IABP) counterpulsation have been implemented in this setting with variable success [4] and the management of cardiogenic shock, refractory to such measures, remains problematic. Ventricular assist devices (VADs) are now an established therapy for cardiogenic shock but their use in the SAH setting has been contraindicated owing to the need for systemic anticoagulation. We present the first successful salvage strategy using VADs in SAH-induced cardiogenic shock. A 42-year-old mother, 4 months post partum, was found unresponsive in the community experiencing tonic-clonic seizures. Cardiorespiratory arrest (pulseless electrical activity) rapidly ensued, and return of spontaneous circulation was achieved after 4 minutes of cardiopulmonary resuscitation. The patient was airlifted to a Accepted for publication June 7, 2013. Address correspondence to Dr Haj-Yahia, Golden Jubilee National Hospital, Glasgow, United Kingdom, G81 4DY; e-mail: hajsaleem@gmail. com.

0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.06.102

Successful use of the total artificial heart in the failing Fontan circulation.

Typical left ventricular assist devices are often ineffective for the failing Fontan circulation. We report the first successful use of a total artifi...
483KB Sizes 1 Downloads 4 Views