Fabrizio De Rita

Reparative surgery of the pulmonary autograft: experience with Ross reoperations

OBJECTIVE Autograft valve and root pathology is the leading cause of Ross procedure failure. To define risk and outcome of autograft valve/root repair at reoperation, a 17-year experience was analysed. METHODS One hundred and thirty-two consecutive late survivors underwent cross-sectional clinical and echocardiographic examination on average 10.8 ± 14.7 years (range 0.4-17) after Ross procedure. Study endpoints were hospital and late morbidity, freedom from autograft reoperation, freedom from root/valve replacement and functional outcome after valve/root repair. RESULTS Twenty-seven (20%) patients underwent 33 cardiac reoperations, the first on average 7.7 ± 4.5 years (range 0.08-16.2) after Ross operation. Nineteen had undergone root replacement, 5 inclusion cylinder and 3 subcoronary grafting. Indication was root pathology in 17 (63%) patients and isolated valve in 10. Surgery consisted in valve repair/sparing in 17 patients and valve/root replacement in 10, with no hospital mortality. Freedom from any autograft valve/root reoperation was 74 ± 5% at 15 years. No patient with valve/root replacement required second reoperation. Instead, 6/17 (35%) patients having autograft valve repair/sparing and followed for 4.2 ± 2.9 years (range 0.3-10.8) required re-repair/AVR, while 11 present mild AI or less. Freedom from autograft valve/root replacement was 83 ± 5% at 15 years. At multivariate analysis, predictors of reoperation were age at Ross (P = 0.002) and use of root technique (P = 0.049). Failure of autograft valve repair/sparing was associated with isolated valve pathology (P = 0.014) and earlier reoperation (P = 0.002). Pre-repair autograft insufficiency was significant at univariate analysis only (P = 0.01). CONCLUSIONS Autograft reoperation carries negligible hospital risk. Pulmonary valve sparing or repair is feasible in half of patients with Ross failure. Concomitant root remodelling and absence of preoperative severe valve dysfunction predict successful and durable repair.

Diagnosis of infection in paediatric veno-arterial cardiac extracorporeal membrane oxygenation: role of procalcitonin and C-reactive protein

OBJECTIVES Plasma concentration of procalcitonin (PCT) and its value in the diagnosis of infection in paediatric patients treated with extracorporeal membrane oxygenation (ECMO) are undefined. This study aimed to define the levels of PCT and C-reactive protein (CRP) in paediatric cardiac ECMO patients and to determine their role in predicting infection, severity of organ dysfunction and clinical outcome. METHODS PCT and CRP plasma concentrations were measured daily in 20 consecutive infants and young children treated with veno-arterial ECMO. Each patient was examined daily for signs of infection and multiple organ dysfunction syndrome (MODS). A total of 139 patient days were classified for infection and MODS. RESULTS The median PCT and CRP plasma concentrations were not increased during infection: 2.4 vs 8.8 ng/ml and 223.8 vs 240.6 mg/l, in patients with vs without infection, respectively. PCT, but not CRP, was significantly elevated during MODS (10.9 vs 1.85 ng/ml) (P = 0.001). The area under the receiver operating characteristic (ROC) curve was 0.984 for PCT (95% confidence interval [CI], 0.962-1.000) compared with 0.347 for CRP (95% CI, 0.211-0.484) (P = 0.001). Only PCT differed significantly in patients weaned from ECMO who survived (2.6 ng/ml) vs patients not weaned from ECMO (10.5 ng/ml) (P = 0.001). The area under the ROC curve was 0.871 (95% CI, 0.786-0.956) compared with 0.261 for CRP (95% CI, 0.145-0.377) (P = 0.001). CONCLUSIONS Neither PCT nor CRP are reliable markers of infection in paediatric cardiac ECMO patients. However, high levels of PCT are associated with MODS. PCT may be used as a prognostic indicator of clinical outcome in this high-risk population.

Rescue extracorporeal life support for acute verapamil and propranolol toxicity in a neonate.

Extracorporeal life support (ECLS) to manage acute antiarrhythmic drugs toxicity in neonates has never been reported. Here presented is a case of venoarterial extracorporeal membrane oxygenation support in a newborn with refractory low cardiac output as a result of acute Ca-channel and β-receptor antagonist toxicity for treatment of paroxysmal supraventricular tachycardia (SVT). Shortly after onset of ECLS, the baby recovered sinus rhythm and subsequent bouts of SVT were controlled by amiodarone infusion and repeated DC shocks. Weaning was possible on the 5th day after implant, once recovery of the left ventricular function and optimization of the antiarrhythmic medication were achieved. In neonates with severe but potentially reversible cardiac dysfunction caused by drug toxicity, ECLS can maintain cardiac output and vital organ perfusion while allowing time for drug redistribution, metabolism, and clearance.

Repair of Congenitally Dysplastic Aortic Valve by Bicuspidization: Midterm Results

BACKGROUND Repair of congenital aortic valve (CAV) lesions may be achieved by creation of either tricuspid or bicuspid valve morphology. To define feasibility and outcome of CAV repair by bicuspidization a 10-year experience was reviewed. METHODS Between January 2002 and December 2011, 147 consecutive patients underwent operation for CAV insufficiency; 58 had valve or root repair (group 1) and 89 had valve or root replacement (group 2). Patients having repair were younger (42.9 vs 51.3 years, p=0.001), with lesser prevalence of severe insufficiency (72% vs 90%, p=0.002). In patients having repair, morphology of CAV was bicuspid in 51, monocuspid in 4, and quadricuspid in 3, whereas in the replacement group it was bicuspid in 87 and quadricuspid in 2 (p=0.04). Surgery consisted of an isolated aortic valve procedure in 20 versus 45 patients, associated with aortic root or ascending aortic repair in 38 versus 44 patients, in group 1 versus 2 (p=0.04). RESULTS There were no hospital and 3 late deaths during a mean follow-up of 3.8±2.5 years (range 0.2 to 10.0). Eight-year survival (89%±10% vs 97%±2% [p=0.7]), freedom from valve-related events (84%±10% vs 89%±4% [p=0.8]), and freedom from aortic valve reoperation (95%±3% vs 93%±3% [p=0.6]) were comparable. Risk factors for reoperation at univariate analysis were isolated valve surgery (p=0.001), Ross operation (p=0.001), and endocarditis (p=0.002). Follow-up echocardiography of repair patients showed mild or less aortic insufficiency in 51 (88%) and mild or less stenosis in 57 (98%). CONCLUSIONS Valve repair by preservation or creation of bicuspid morphology is feasible in almost half of all comers with CAV insufficiency, with satisfactory and stable midterm functional outcome. Rates of valve-related adverse events and reoperation are similar to those of patients having replacement.

An alternative method for neonatal cerebro-myocardial perfusion.

Several techniques have already been described for selective cerebral perfusion during repair of aortic arch pathology in children. One method combining cerebral with myocardial perfusion has also been proposed. A novel technique is reported here for selective and independent cerebro-myocardial perfusion for neonatal and infant arch surgery. Technical aspects and potential advantages are discussed.

Comparison Between D901 Lilliput 1 and Kids D100 Neonatal Oxygenators: Toward Bypass Circuit Miniaturization

Progress in biomaterial technology and improvements in surgical and perfusion strategy ameliorated morbidity and mortality in pediatric cardiac surgery. In this study, we describe our clinical experience comparing performance of two neonatal oxygenators. From January 2002 to March 2011, 159 infants with less than 5 kg body weight underwent heart surgery. Ninety-four patients received a D901 Lilliput 1 oxygenator with standard bypass circuit (group A), while 65 received a D100 Kids with miniaturized bypass circuit (group B). Miniaturization consisted in shortened arterial, venous, cardioplegia, and pump-master lines. Priming composition consisted in Ringers acetate solution with addition of albumin and blood, with target hematocrit of 24% or greater. In group B cardiopulmonary bypass (CPB) was vacuum-assisted and started with an empty venous line. Modified ultrafiltration and Cell-Saver blood infusion was routinely applied in both groups. Average ± standard deviation (SD) age at repair was 37 ± 38 days in group A and 59 ± 60 days in group B (P = 0.005). Average ± SD weight, height, and body surface area were 3.5 ± 0.7 kg, 52 ± 4 cm, and 0.22 ± 0.03 m(2) , respectively, in group A, and 3.7 ± 1 kg, 53 ± 5 cm, and 0.23 ± 0.02 m(2) , respectively, in group B (P = not significant [NS]). Male sex was predominant (55 vs. 58%, P = NS). Priming volume was 524 ± 67 mL (group A) and 337 ± 53 mL (group B) (P = 0.001). There were no statistical differences in hemoglobin at the start, during, and at the end of CPB, but group A required higher blood volume added to the prime (111 ± 33 vs. 93 ± 31 mL, P = 0.001). In group B, two surgical procedures were completed in total hemodilution. In group B, CPB time and aortic cross-clamp time were shorter than in group A (106 ± 52 vs. 142 ± 78 min and 44 ± 31 vs. 64 ± 31 min, respectively, P = 0.001). There were 16 hospital deaths in group A and 4 in group B (P = 0.04). Durations of mechanical ventilation and intensive care unit stay were 5.3 ± 3.2 vs. 4.1 ± 3.2 days (P = 0.02) and 6.5 ± 4.9 vs. 5.1 ± 3 days (P = 0.03), respectively. There were significant differences in inotropic score (1083 ± 1175 vs. 682 ± 938, P = 0.04) and blood postoperative transfusion (153 ± 226 vs. 90 ± 61 mL, P = 0.04). Twenty-seven patients in group A and 10 in group B presented with major adverse postoperative complications (P = 0.04). Use of neonatal oxygenators with low priming volume, associated with a miniaturized bypass circuit, seems to be a favorable strategy to decrease postoperative morbidity after cardiac surgery in neonates and infants.

Current management of double-outlet left ventricle: towards biventricular repair in infancy.

Background Double-outlet left ventricle (DOLV) is an extremely rare type of ventriculoarterial connection, mostly reported anecdotally with wide variations in morphology, surgical strategy and clinical outcome. The aim of the present study was to define current morbidity and mortality of biventricular repair and discuss surgical management in infancy. Methods Inclusion criteria were English literature publication; balanced ventricular development and completion of biventricular repair. Institutional experience was also reported. Study end points were survival and freedom from cardiovascular events. Univariate analysis to identify risk factors for complications was performed. Results During a 45-year interval, 37 cases of DOLV were identified. Median age at repair was 48 months (range 0.3–336); seven patients were infants. Extracardiac repair was performed in 21 (57%) patients, right ventricular outflow patch in seven, pulmonary translocation in six, and intraventricular repair in three. There were three (8%) hospital deaths, due to right heart failure, and seven (21%) patients experienced 10 perioperative complications. Univariate analysis showed year of operation (P = 0.02) and outflow patch technique (P = 0.003) as risk factors for hospital morbidity and mortality. During a median follow-up of 26 months (range 1–192), there was one death and seven reoperations. Ten-year survival and freedom from adverse cardiovascular events were 87 ± 6 and 70 ± 10%, respectively. Conclusion Although seldom reported in the past, repair of DOLV in infancy does not increase surgical risk. Early and late morbidity are mostly due to right heart complications and more common after right ventricular outflow patch repair. Paucity of follow-up information may underestimate actual late adverse event rate.

Hot-Air Balloon Explosion in the Heart

A 16-year old girl experiencing palpitations underwent elective transthoracic echocardiographic examination at a nearby clinic, which showed a 3-cm balloon-like, cystic structure, with inhomogeneous content, adherent to the atrial septum and anchored above the anteroseptal commissure of the tricuspid valve without evidence of flow from the aorta to the right atrium (Figure 1A). On examination, blood pressure (120/70 mm Hg) and heart rate (70 bpm) were normal, but she was advised to limit physical activity. While waiting for scheduled MRI scan, she was admitted to the emergency department for acute vision loss and syncope. At physical examination, the patient presented dyspnea, hypotension (90/50 mm Hg), and tachycardia (130 bpm); a 5/6 continuous, rough murmur was heard over the left precordium. Chest x-ray revealed cardiomegaly and pulmonary congestion. To refine diagnosis of the cardiac mass, the girl underwent transesophageal echocardiographic examination, which demonstrated communication between the aortic root and the previously identified cystic mass, now ruptured in …

Two Cases of Double-Outlet Left Ventricle Detected Prenatally

Double-outlet left ventricle is an exceedingly rare congenital heart defect. Its prenatal detection and precise anatomical definition are challenging for a variety of reasons and have never been previously reported. Here described are 2 cases of prenatally diagnosed double-outlet left ventricle. The technical limitations of prenatal diagnosis and its implications for the surgical management of patients affected by such a rare condition are discussed.