Tetralogy of Fallot (TOF) is a congenital heart disease frequently treated by surgical repair to relieve symptoms and improve survival. However, despite the performing of an optimal surgical repair, TOF patients are at times characterized by a poor long-term survival rate, likely due to cardiac causes such as ventricular arrhythmias, with subsequent sudden death. In the 80s it was irrefutably demonstrated that QRS prolongation ≥180 msec at basal electrocardiogram is a strong predictor for refining risk stratification for ventricular tachycardia in these patients. The aim of this research was to undertake a review of all studies conducted to assess the impact of QRS duration on the development of life-threatening ventricular arrhythmias in repaired TOF subjects.

Background

Previous Italian paediatric blood pressure (BP) tables overestimated the prevalence of hypertension in adolescents of specific geographic areas, such as Sardinia, an island in the Mediterranean Sea. This is probably due to a not very homogeneous distribution of the subjects studied, most from Middle and Northern Italy, and the long period from the survey.

Methods

BPs were repeatedly measured over a period of 3 years in 839 children (52.6% males. Age range: from 11 to 14 years during this period), using a standard mercury sphygmomanometer. For each gender, the specific percentile curves of systolic and diastolic BP were constructed.

Results (corrected by the 50th percentile of height)

Males (11-14 years)

mean systolic BP (50th centile): from 111 to 115 mmHg. Hypertensive systolic BP (> 95th percentile): from 127 to 135 mmHg. Mean diastolic BP (50th centile): from 65 to 69 mmHg. Hypertensive diastolic BP (> 95th percentile): from 78 to 82 mmHg.

Females(11-14 years)

mean systolic BP (50th centile): from 110 to 112 mmHg. Hypertensive systolic BP (> 95th percentile): from 127 to 130 mmHg. Mean diastolic BP (50th centile): from 65 to 67. Hypertensive diastolic BP (> 95th percentile): from 78 to 80 mmHg.

Conclusions

Sardinian BP tables emphasizes the need to integrate the previous standards with more up-to-date and representative reports on Italian children, as periodically performed in the USA, in order to increase the number of subjects to be checked, and to obtain a national coverage better and more completely representative of every geographic area of our country.

Introduction

Morgagni's congenital diaphragmatic defect is a rare malformation, the diagnosis of which, as in our case report, may be problematic. To the best of our knowledge, this is the first report of this kind of hernia presenting with signs and symptoms of severe cardiac malformation.

Case presentation

We report the case of a three-month-old Caucasian baby boy, who presented with heart failure and severe pulmonary hypertension. Compression of the heart by a bowel loop in the chest led to an incorrect diagnosis of congenital heart disease.

Conclusions

Even in this era of highly sophisticated diagnostic tools, a simple radiograph can provide sufficient information for a precise, rapid diagnosis.

Background

Alport syndrome (AS) is a rare inherited disorder characterized by an inflammation of the kidneys and damage to the glomerular capillaries, ultimately leading to renal failure at an early age. To date, rare reports of cardiac involvement in AS have been described, due in the majority of cases to the higher risk of heart conduction abnormalities in these patients, at times requiring implantation of a transcutaneous pacemaker. An increased risk of hypertension is likewise commonly featured.

Case presentation

We report the case of a 17-year-old female affected by a very severe early form of AS. A previously unreported association of the syndrome with congenital heart disease (CHD), (in this case membranous ventricular septal defect), is also reported. A possible pathophysiological mechanism underlying the concomitant manifestation of these two disorders is suggested. Complications implicated in surgical treatment of CHD are described. Clinical and therapeutic management of AS with cardiovascular involvement are discussed, and a short literature review performed.

Conclusions

This first report of a cardiovascular association highlights the possible involvement of collagen mutations in the two pathologies. Even when drug-resistance appears to be responsible for the failure to control secondary hypertension in AS, clonidine may represent a safe, effective option in the normalization of high blood pressure.

Cor triatriatum is a rare congenital heart defect characterized by the presence of a fibromuscular membrane dividing the left atrium into two distinct chambers. Lipomatous hypertrophy of the atrial septum is an infrequently observed benign abnormality caused by large fatty tissue deposits in the interatrial septum. An increased incidence of atrial arrhythmias is described in both pathologies, while a significant obstruction of blood flow mimicking mitral stenosis is typically manifested in cor triatriatum. We report the case of a 75-year-old woman with a previously undescribed association of the above stated abnormalities detected by both transthoracic and transeosophageal echocardiography. Diagnosis was confirmed by means of computed tomography. The singular physiologic and anatomic factors underlying survival until such a late age are described. The diagnostic, therapeutic and surgical management is discussed and a short review of the literature performed.