MCS XXXX-XXXX 2000-6993 Mechanical Circulatory Support Vol. 2, No. 0, March 2011, pp. 1–4 sici xxx PUBMED Abbreviation Antonio di Coste

*Antonio di Coste via Monopoli 65 70013 Castellana Grotte Italy +393387908129

Vincenzo Cassano Dario Troise Francesco Cassano Andrea Marzullo Gilda Caruso Cosima Lasaracina Michael S. Firstenberg Francesco P. Annecchino Division of Paediatric Cardiac Surgery Pope Giovanni XXIII Hospital

Bari Italy

University Department of Pathology Policlinico di Bari Hospital

Bari Italy

University Department of Cardiology Policlinico di Bari Hospital

Bari Italy

Division of Cardiac Surgery The Ohio State University Medical Center

Columbus Ohio

Mechanical Circulatory Support PATIENT EXPERIENCE LVAD to recovery heart failure in children We present a case of a 7-year-old, 20 kilogram male child, admitted in our Center with a diagnosis of fulminant myocarditis with a large pericardial effusion and severe hemodynamic instability. Due to failure of medical therapy, a left ventricular assistant device (LVAD) was implanted. After 4 days, the patient was successfully weaned from mechanical support. Myocardial biopsy showed evidence of acute viral myocarditis. Our experience suggests that the left ventricular assist device therapy might offer circulatory support in pediatric cases of fulminant myocarditis. fulminant myocarditis mechanical circulatory support heart failure Bacterial myocarditis is extremely rare with most cases caused by viral infections. Patients are often asymptomatic until they present with acute decompensated heart failure. Other symptoms secondary to low cardiac output and infection include fever, abdominal pain, tachycardia, and occasionally symptoms suggestive of an upper airways infection may be present and may cloud the diagnosis. Most cases of viral myocarditis are caused by Coxsackie virus.1 Chronic myocarditis is typically an advanced process and is associated with heart failure and myocardial fibrosis.2 We present a case of a 7-year-old child who presented with acute heart failure in whom temporary treatment with a ventricular assistance device (VAD) assisted in complete recovery. A 7-year-old, 20 kilogram, male child was referred to our Center with clinical signs of fever, abdominal pain, asthenia, and periorbital edema. Symptoms started 6–7 days before admission. Upon admission, the heart rate was 170 bpm with a sinus tachycardia, cyanosis, anemia, metabolic acidosis (pH = 7.1, PO₂=65 mmHg, P_CO2=45 mmHg), oliguria, and weak pulses with systolic pressure of 60 mmHg. There was also lethargy probably due to cardiogenic shock. Minimal response was seen with the administration of Dobutamine (5 mcg/kg/min), Milrinone (0.5 mcg/kg/min), Furosemide (1 mg/kg/hr), and Adrenaline (0.07 mcg/kg/min). Mechanical ventilation was required due to respiratory failure.3 4 The echocardiogram showed an ejection fraction of 10% with severe hypokinesia and biventricular failure, a mild–severe pericardial effusion, and low–mild dilatation of the left ventricle.5 The chest roentgenogram showed cardiac failure (Fig. 1). Cardiac Troponin I (14 ng/ml) and creatine phosphokinase (CPK-MB = 589 ng/ml) levels were elevated. Due to failure of medical therapy and a worsening clinical picture, we decided to treat the patient with a left ventricular assistance device (Sorin Revolution®, Sorin Group USA, Inc., Arvada, CO) from the left atrium to ascending aorta via median sternotomy. Pacifico 24 (Medtronic Inc. Minneapolis, MN, USA) cannulas were used for venous outflow access and a DPL 12 (Medtronic Inc. Minneapolis, USA) was used for aortic inflow (Fig. 2). Furthermore, the patient required hemodialysis for persistent oliguria due to a renal acute failure. The myocardial biopsy confirmed a viral etiology with a myocardial interstitial lymphoplasmatic infiltration positive to CD 45 Lc, CD 79a, and CD 3 with fibrosis areas.6 Broad-spectrum antibiotics and intravenous immunoglobulin therapy were also initiated. After 4 days of circulatory support, we were able to completely wean from the LVAD support. At the time of decannulation, the ejection fraction had returned to normal (EF = 74% by echo). At 1-year post-LVAD explant the patient is well, leading a normal life, and without the need for any medications (Fig. 3).

Chest X-rays at admission in hospital.

Chest X-ray demonstrating aortic and right atrial LVAD cannulas.

Chest X-ray, 1-year follow-up.

Fulminant postviral myocarditis often presents suddenly in otherwise previously healthy patients. Patients can present in acute heart failure with left ventricular dilatation and a severely depressed ejection contraction. Unlike many forms of heart failure (i.e. ischemic or complex congenital), myocarditis may result in a self-limiting heart failure and provided patients do not die from severe multi-organ failure and sepsis from cardiac shock, recovery can occur. As demonstrated in our case, LVAD support may be useful to support the physiology until cardiac recovery can occur7 8 (Table 1). In cases in which myocardial recovery does not occur, the patient may be considered for cardiac transplantation. Fortunately, no cerebral vascular or neurologic complications occurred in our patient as this is often encountered with pediatric mechanical support such as extra-corporeal membrane oxygenation (ECMO).9 Furthermore, once cardiac recovery occurred, the patient also slowly recovered renal function. He was eventually weaned from dialysis.10 The patient was also weaned from the ventilator without difficulty within several days. All of the problems are often associated with temporary mechanical support and close attention to details can prevent associated severe complications. In our experience, treatment of acute cardiac failure with mechanical circulatory support of acute left ventricular failure in a small 7-year-old child due to a postviral fulminant myocarditis was safe and successful. As these patients have isolated cardiac failure with preserved oxygenation support with an LVAD instead of cardiopulmonary support with an ECMO can be considered. This helps to confirm the important role of this treatment option in the pediatric age group. Consistent with the literature, early implementation of mechanical support is critical in avoiding irreversible end-organ damage and the acute (vs chronic) clinic deterioration may also be a favorable prognostic indicator for a similar rapid recovery.11 In our experience, support of left ventricular failure with a LVAD has been successful in small and young children. Consistent with the literature, medical refractory fulminant acute myocarditis can be supported with mechanical support options such as ECMO, intra-aortic balloon counterpulsation (IABP), or – as in our case – implantable left ventricular assist devices. Early implementation, prior to the onset of irreversible end-organ or neurologic damage is critical in potentially salvaging these critically ill patients. There is no conflict of interest in the present study for any of the authors. Matsue Y Kumasaka L Nagahori W Ohno M Suzuki M Matsumura A A case of fulminant myocarditis with three recurrences and recoveries Int Heart J 2010 51 218 19 Baughman KL. Diagnosis of myocarditis: death of Dallas criteria Circulation 2006 113 593 95 Oda T Yasunaga H Tsutsumi Y Shojima T Zaima Y Nishino H A child with influenza A (H1N1) – associated myocarditis rescued by extracorporeal membrane oxygenation J Artif Organs 2010 13 232 34 Hu W Mao S Hu W Zhu Y Liu C. ECMO support in a child with acute fulminant myocarditis Indian J Pediatr 2010 1 1 4 Felker GM Boehmer JP Hruban RH Hutchins GM Kasper EK Baughman KL Echocardiographic findings in fulminant and acute myocarditis J Am Coll Cardiol 2000 36 227 32 Brucato A Colombo T Bonacina E Orcese C Vago L Oliva F Fulminant myocarditis during HIV seroconversion: recovery with temporary left ventricular mechanical assistance Ital Heart J 2004 5 228 31 El-Banayosy NR Arusoglu L Kleikamp G Minami K Körfer R. Recovery of organ disfunction during bridging to heart transplantation in children and adolescents Int J Artif Organs 2003 26 395 400 Morales DL Zafar F Rossano JW Salazar JD Jefferies JL Graves DE Use of ventricular assist devices in children across the United States: analysis of 7.5 million pediatric hospitalizations Ann Thorac Surg 2010 90 1313 18 Tsukui H Abla A Teuteberg JJ McNamara DM Mathier MA Cadaret LM Cerebrovascular accidents in patients with a ventricular assist device J Thorac Cardiovasc Surg 2007 134 115 23 Sandner SE Zimpfer D Zrunek P Rajek A Schima H Dunkler D Renal function and outcome after continuous flow left ventricular assist device implantation Ann Thorac Surg 2009 87 1072 78 Oshima K Kunimoto F Hinohara H Hayashi Y Hirato J Tajima Y Fulminant myocarditis treated with percutaneous cardiopulmonary support system (PCPS) Ann Thorac Cardiovasc Surg 2008 14 75 80