Papers by Fernando Picchio
Pediatric Cardiology, Jun 1, 1982
PubMed, Nov 1, 2021
Pediatric cardiology has matured profoundly over the last 50 years, paralleled by a similar devel... more Pediatric cardiology has matured profoundly over the last 50 years, paralleled by a similar development in pediatric cardiac surgery and cardiac anesthesia. This field of medicine provides structured and effective care for a very heterogeneous group of diseases including congenital heart disease, cardiomyopathies and heart failure, myocarditis, rheumatic heart disease, inherited and acquired arrhythmias, Kawasaki disease and more recently multisystemic inflammatory syndrome of children related to primary infection by SARS-CoV-2. This review summarizes achievements and results in selected topics of pediatric cardiology and cardiac surgery with focused attention to the diagnosis and management of congenital heart diseases.
![Research paper thumbnail of [Transposition of the great arteries: anatomic types of left ventricular outflow tract obstruction (author's transl)]](https://a.academia-assets.com/images/blank-paper.jpg)
PubMed, 1979
A postmortem investigation has been carried out in 48 heart specimens with transposition of the g... more A postmortem investigation has been carried out in 48 heart specimens with transposition of the great arteries (TGA) to evaluate incidence and type of left ventricular outflow tract obstruction. The frequence of pulmonary stenosis in our series (23%) is similar to those reported from previous studies. Cases have been divided in two groups according to presence or absence of ventricular septal defect. In cases with intact ventricular septum the obstruction was determined by asymmetric hypertrophy of the septum (1 observation) and by abnormal attachement of clefted anterior mitral leaflet to the interventricular septum (2 observations). In the specimens with ventricular septal defect, 4 presented a stenosis due to malalignment of the infundibular septum, associated with valvular stenosis in 1 case and with valvular stenosis and fibrous subvalvular ring in 2. Another case presented a stenotic pulmonary valve and a parachute mitral valve. The remaining 3 showed a cleft of the anterior leaflet of the mitral valve. The high incidence of infundibular malalignment obstruction is underlined together with its association with A-P or L-position of the aorta. This peculiar relation of the great arteries could be angiocardiographically employed as indicative for the presence of this type of left outflow tract obstruction in TGA.

Journal of Cardiovascular Medicine, 2007
During the past three decades, interventional cardiology and cardiac surgery have found solutions... more During the past three decades, interventional cardiology and cardiac surgery have found solutions even for the most complex congenital heart malformations with an overall low operative mortality A careful clinical and instrumental follow-up of postoperative congenital heart disease patients is fundamental not only to prevent complications and/or to treat eventual residua and sequelae, but also to modify future surgical strategies on the basis of long-term results. To be able to give a correct prognostic meaning to the data collected during the follow-up, the cardiologist should have an excellent knowledge of the native defect, the surgical technique and the post-surgical anatomy and physiology. Major cardiological concerns during a follow-up after corrective surgery are: arrhythmias; heart failure; cyanosis and erythrocytosis; and infective endocarditis. Psychosocial needs, such as employment, contraception, pregnancy and physical exercise, are very important to enable a 'normal' life, complying with the postoperative hemodynamic situation of the patients.
European Heart Journal, Nov 14, 2007
Common variants in myocardial ion channel genes modify the QT interval in the general population:... more Common variants in myocardial ion channel genes modify the QT interval in the general population: results from the KORA study.
Pediatric Cardiology, 1992
A 9-year-old patient who had had a syncope was found to have atrial flutter in a resting electroc... more A 9-year-old patient who had had a syncope was found to have atrial flutter in a resting electrocardiogram (ECG). Brief phases of sinus arrest had previously occurred after drug conversion to sinus rhythm. Structural heart disease was excluded by cardiac catheterization and angiography. Electrophysiologic study revealed a sick sinus syndrome, associated with diffuse impairment of the conduction system (supra-, infra-, and intrahisian block). Epimyocardial and an endocardial pacemaker implantation failed because of high stimulation threshold, after 3 years and 2 weeks, respectively. At the third implantation a steroid-eluting endocardial pacing lead was used and satisfactory pacing was still present 2 years later.

PubMed, Feb 1, 2002
Background: Many devices currently used for the closure of the patent ductus arteriosus are claim... more Background: Many devices currently used for the closure of the patent ductus arteriosus are claimed to be safe and cost-effective, but only few data exist with respect to the gold standard of the Rashkind occluder. The aim of this study was to assess the efficacy and safety of three new different devices and to compare the results to those of a control group of patients carrying a Rashkind occluder. This should provide the basis for further cost-analysis studies. Methods: The records of all patients who underwent closure of the patent ductus at our Institution from April 1989 to May 2001 were reviewed. Eighty patients (median age 10.3 years, median weight 27.6 kg) were treated (25 with a Rashkind device, 11 with Duct-Occlud coils, 35 with Cook detachable coils, 9 with the Amplatzer system). Results: Kaplan-Meyer estimates of long-term complete occlusion of the ductus showed, compared to the Rashkind device, a significant improvement for the Cook and Amplatzer (p = 0.025 and p = 0.003, respectively) devices but not for the Duct-Occlud coils (p = 0.165). One patient of the Duct-Occlud group (9%) and 3 with the Rashkind device (12%) featured a significant residual shunt and needed a second intervention. The complication rate was 4% for the Rashkind occluder, 5% for the Cook coils, 9% for the Duct-Occlud system, and 11% for the Amplatzer device (p > 0.05). Conclusions: The new devices are as safe as the Rashkind occluder and provide effective treatment. The Cook coils and the Amplatzer occluder offer better results compared to the Rashkind and Duct-Occlud devices.

PubMed, Jun 1, 2001
Background: The aim of this study was to verify if surgery is beneficial for patients older than ... more Background: The aim of this study was to verify if surgery is beneficial for patients older than 50 years. Methods: Sixty-five patients older than 50 years were operated for a secundum atrial septal defect between November 1974 and November 1998. Preoperative data were obtained from hospital records; postoperative data from written questionnaires or direct telephone interviews. A comparison of pre and postoperative data was possible in 53 patients. Results: The operative mortality was 0%. One patient died of a thromboembolic complication 32 days after surgery. The mean follow-up was 9 +/- 6 years. After surgery, clinical improvement occurred in 22 patients (41.5%) with the majority of them (69.8%) being asymptomatic or only mildly symptomatic. The occurrence of atrial fibrillation/flutter did not decrease after surgery (39.6 vs 26.4%). A thromboembolic event occurred in 2 patients before surgery and in 2 patients postoperatively; all of them had supraventricular arrhythmias and were not taking anticoagulants. Conclusions: Surgical closure of atrial septal defects in patients older than 50 years is feasible. The mortality is low. In this age group, surgery has a beneficial effect on the clinical status of the patients but not on the occurrence of supraventricular arrhythmias that can affect morbidity and mortality in patients who are not treated with anticoagulants.

The Annals of Thoracic Surgery, Mar 1, 2006
Background. Genetic syndromes occur in 20% of patients with tetralogy of Fallot (TOF). The impact... more Background. Genetic syndromes occur in 20% of patients with tetralogy of Fallot (TOF). The impact of genetic syndromes on surgical repair of TOF in infancy is still under investigation. Methods. This retrospective study reviews the outcome of 306 consecutive patients (median age, 5.1 months) who underwent primary (266) or staged (40) repair of TOF between 1994 and 2004. Total follow-up was 1,188 patient-years (mean, 57 months). Results. Genetic syndromes were documented in 85 patients (27.8%), including 22q11 deletion (27), trisomy 21 (13), vertebral, anal, cardiac, tracheoesophageal, renal, and limb abnormalities (VACTERL, 12), and others (33). Hypoplastic pulmonary arteries (PA) were more common in syndromic (19/85 ؍ 22.3%) than nonsyndromic TOF (20/221 ؍ 9.04%) (p < 0.001). Primary repair was performed in 82.4% syndromic and 88.7% nonsyndromic TOF (p ؍ not significant [NS]). Ten-year actuarial survival was 94.1 ؎ 2.3% in nonsyndromic and 84.3 ؎4.2% in syndromic TOF (p < 0.001). Ten-year survival was 96.3 ؎ 3.6% for del22q11, 100% for trisomy 21, 63.6 ؎ 14.5% for VACTERL, and 78.5 ؎ 7.3% for patients with other syndromes (p ؍ 0.022). Survival in syndromic TOF with normal PA anatomy was 89.6 ؎ 4.2% for primary repair and 85.7 ؎ 12.8% for staged repair (p ؍ NS); freedom from reoperation after complete repair was 74.4 ؎ 6.4% for primary correction and 56.3 ؎ 11.9% for staged repair (p ؍ 0.04). Cox proportional hazard identified the presence of genetic syndrome (p ؍ 0.011) and central PA hypoplasia (p ؍ 0.002) as independent predictors of mortality. Conclusions. Pulmonary arborization defects and genetic syndromes other than del22q11 or trisomy 21, are associated with worse outcome after correction of TOF. Primary TOF repair in syndromic patients with normal PA anatomy is a valid surgical strategy, with no additional risk for mortality and higher freedom from reintervention.

Drugs, 2008
ABSTRACT A large proportion of patients with congenital heart disease (CHD), in particular those ... more ABSTRACT A large proportion of patients with congenital heart disease (CHD), in particular those with relevant systemic-to-pulmonary shunts, will develop pulmonary arterial hypertension (PAH) if left untreated. Persistent exposure of the pulmonary vasculature to increased blood flow, as well as increased pressure, may result in pulmonary obstructive arteriopathy, which leads to increased pulmonary vascular resistance that, if it approaches or exceeds systemic resistance, will result in shunt reversal. Eisenmenger&#39;s syndrome, the most advanced form of PAH associated with CHD, is defined as CHD with an initial large systemic-to-pulmonary shunt that induces severe pulmonary vascular disease and PAH, with resultant reversal of the shunt and central cyanosis. The histopathological and pathobiological changes seen in patients with PAH associated with congenital systemic-to-pulmonary shunts, such as endothelial dysfunction of the pulmonary vasculature, are considered similar to those observed in idiopathic or other associated forms of PAH. A pathological and pathophysiological classification of CHD with systemic-to-pulmonary shunt leading to PAH has been developed that includes specific characteristics, such as the type, dimensions and direction of the shunt, extracardiac abnormalities and repair status. A clinically oriented classification has also been proposed. The prevalence of PAH associated with congenital systemic-to-pulmonary shunts in Western countries has been estimated to range between 1.6 and 12.5 cases per million adults, with 25-50% of this population affected by Eisenmenger&#39;s syndrome. Clinically, Eisenmenger&#39;s syndrome presents with multiple organ involvement, with progressive deterioration of function over time. The signs and symptoms of Eisenmenger&#39;s syndrome in the advanced stages include central cyanosis, dyspnoea, fatigue, haemoptysis, syncope and right-sided heart failure. Survival of patients with Eisenmenger&#39;s syndrome is clearly less than that of the general population, but appears to be better than that of patients with idiopathic PAH in a comparable functional class. The treatment strategy for patients with PAH associated with congenital systemic-to-pulmonary shunts and, in particular, those with Eisenmenger&#39;s syndrome is based mainly on clinical experience rather than being evidence based. General measures include recommendations for physical activity, pregnancy, infections, air travel, exposure to high altitudes and elective surgery, and that psychological assistance be provided as necessary. Phlebotomies are required only when hyperviscosity of the blood is evident, usually when the haematocrit is &gt;65%. The use of supplemental oxygen therapy is controversial and it should be used only in patients in whom it produces a consistent increase in arterial oxygen saturation. Oral anticoagulant treatment with warfarin can be initiated in patients with pulmonary artery thrombosis and absent, or only mild, haemoptysis. The following three classes of drugs targeting the correction of abnormalities in endothelial dysfunction have been approved recently for the treatment of PAH: (i) prostanoids; (ii) endothelin receptor antagonists; and (iii) phosphodiesterase-5 inhibitors. The efficacy and safety of these compounds have been confirmed in uncontrolled studies in patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts, as well as in patients with Eisenmenger&#39;s syndrome. One randomized controlled trial reported favourable short- and long-term outcomes of treatment with the orally active dual endothelin receptor antagonist bosentan in patients with Eisenmenger&#39;s syndrome. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation are options for Eisenmenger&#39;s syndrome patients with a poor prognosis. A treatment algorithm based on the one used in the treatment of PAH patients is proposed for patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts and Eisenmenger&#39;s syndrome.
International Journal of Cardiology, Nov 1, 2010
Amiodarone is an effective antiarrhythmic agent and represents the drug of choice in the treatmen... more Amiodarone is an effective antiarrhythmic agent and represents the drug of choice in the treatment of severe arrhythmias, especially in the setting of ventricular dysfunction. Amiodarone has the potential for interaction with many cardiac and non-cardiac drugs. Nonetheless few incompatibilities have been reported. We report the incompatibility between amiodarone and heparin administrated in the same vein in a case of a one month old baby with atrial flutter. This topic needs more attention, due to the frequent co-administration of these two drugs in tachyarrhytmias with high thromboembolic risk.
American Journal of Medical Genetics, 2005
Tricuspid atresia (TriAt), the third most common cyanotic congenital heart defect (CHD), consists... more Tricuspid atresia (TriAt), the third most common cyanotic congenital heart defect (CHD), consists of complete lack of tricuspid valve formation, with no connection between the right atrium and the right ventricle. To date, the genetic mechanism responsible of TriAt is still obscure. However, animal models have suggested a role of cardiogenic Zfpm2/Fog2 and Hey2 genes in the pathogenesis of TriAt. Therefore, we screened 40 individuals affected by nonsyndromic TriAt for ZFPM2/FOG2 and HEY2 gene mutations. No pathogenetic mutation has been identified, thus failing to demonstrate a major role of ZFPM2/FOG2 and HEY2 genes in the pathogenesis of human TriAt.
Pediatrics, Feb 1, 2004
Posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and car... more Posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, and cardiac defects and eye abnormalities (PHACE) is a rare congenital anomaly with a broad spectrum of clinical manifestations with a striking female predominance. We describe an infant with PHACE anomaly and aortic coarctation who underwent cardiac catheterization to clearly define the complex anatomy of the aortic lesion before surgical repair. Review of the literature documents a highly significant association between ipsilateral hemangiomas and cerebrovascular and aortic arch anomalies. We conclude that cardiac catheterization is required to define the complex anatomy of aortic lesions and should be performed in all patients with PHACE and evident aortic involvement to plan appropriate and safe surgical repair.

The Annals of Thoracic Surgery, Aug 1, 2004
Background. Several studies have shown that Down's syndrome is not a risk factor for biventricula... more Background. Several studies have shown that Down's syndrome is not a risk factor for biventricular repair of complete atrioventricular septal defects. However, few data are available about the comprehensive outcome of all the cardiac surgical procedures in patients with trisomy 21, including palliative surgery. Methods. This is a retrospective study of 206 consecutive patients who underwent cardiac surgery from January 1992 to January 2002. Data about mortality and morbidity were analyzed and the impact of Down's syndrome was evaluated. Results. Overall mortality was 7.7%. Actuarial survival was 94% among patients with Down's syndrome versus 86% of the group with normal karyotype (p ؍ 0.12). The presence of unbalanced ventricles was the only independent risk factor affecting survival at multivariate analysis (p < 0.0001). The need for a Norwood type surgery was more frequent among non-Down patients (12.0% vs 1.5%, p ؍ 0.02) as was the prevalence of pulmonary artery banding operations (22.9% vs 9.3%, p ؍ 0.04). Cumulative mortality after palliation was higher in non-Down patients (44% vs 2.9%, p ؍ 0.0001). Freedom from reoperation was lower in the group with normal chromosomes in respect to patients with Down's syndrome (81.4% vs 94.6%, p ؍ 0.04), due to the higher prevalence of anomalies of the mitral valve (4.9% vs 1.8%, p ؍ 0.03) or left ventricular outflow tract (7.3% vs 0%, p ؍ 0.01). Conclusions. Down patients showed a decreased risk for biventricular repair and lower mortality and morbidity in cases of complex cardiac malformations requiring complex palliative operations.

Journal of the American College of Cardiology, Apr 1, 2009
The goal of this study was to assess the prognostic value of the cardiopulmonary exercise test (C... more The goal of this study was to assess the prognostic value of the cardiopulmonary exercise test (CPET) in patients who received a Mustard and Senning (M/S) operation. Background Patients who received an M/S operation have increased long-term risk of cardiovascular morbidity and mortality. Limited information is available on how to stratify risk in this population. Methods Between 1996 and 2007, 274 adults (age 26.3 Ϯ 8.9 years, range 16 to 50 years) who had received a Mustard (n ϭ 144) or Senning (n ϭ 130) operation in infancy were studied with CPET. During a follow-up of 3.9 Ϯ 2.3 years (range 0.2 to 10.8 years), 12 patients died at an age of 36 Ϯ 14 years, and 46 patients required a cardiac-related emergency (Ͻ24 h from the onset of symptom/condition) hospital admission at an age of 30 Ϯ 11 years. Results At multivariate Cox analysis, the slope of ventilation per unit of carbon dioxide output (VE/VCO 2 slope) (hazard ratio: 1.088, p Ͻ 0.0001) and percentage of predicted peak oxygen uptake (VO 2 %) (hazard ratio: 0.979, p ϭ 0.0136) were the strongest predictors of death/cardiac-related emergency hospital admission among demographic, clinical, and exercise variables. A VE/VCO 2 slope Ն35.4 (hazard ratio: 10.7, 95% confidence interval [CI]: 7.8 to 24.6), and a peak VO 2 % Յ52.3% (hazard ratio: 3.4, 95% CI: 2.5 to 8.2) were associated with an increased 4-year risk of death/cardiac-related emergency hospital admission. Patients who had both a VE/VCO 2 slope Ն35.4 and a peak VO 2 % Յ52.3% of predicted value were at highest risk (4-year event rate: 78.8%). Conclusions CPET provides important prognostic information in adults with M/S operation. Subjects with enhanced ventilatory response to exercise or those with poor exercise capacity have a substantially higher 4-year risk of death/ cardiac-related emergency hospital admission.
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Papers by Fernando Picchio