Extreme pulsus alternans with P wave alternans in a child.

Pulsus alternans was observed in a child with left ventricular dysfunction secondary to systemic hypertension. The Echo-Doppler of supravalvular aorta and a simultaneous electrocardiographic trace (ECG) clearly demonstrated the mechanism of an apparent abrupt halving of pulse frequency compared with the ECG, similar to an electromechanical dissociation 2:1. Besides the extreme pulsus alternans, a P wave and a PP cycle length change were also observed. Both mechanical and electrical alternans disappeared with afterload reduction by nitroprusside treatment.

[Manual obliteration of femoral artery pseudoaneurysm. Report of a case and brief review of the literature]. / Obliterazione manuale di uno pseudoaneurisma dell'arteria femorale. Presentazione di un caso con breve rassegna della letteratura.

The authors report a case of non-surgical repair of a femoral artery pseudoaneurysm due to percutaneous catheterization. The procedure was performed by direct manual compression under intermittent color-flow duplex ultrasound control. Some conditions reported in the Literature as unfavorable for a prompt resolution were present (long time interval between invasive procedure and treatment, anticoagulant medication); nevertheless the manoeuvre was successful after only 20'. In comparison with previous experiences (reporting direct compression with the ultrasound transducer or with a mechanical vascular clamp device) the authors underline some observations: a) direct manual compression seems to guarantee a better stability in the optimal position allowing flow interruption in the lesion; b) ultrasound guide is necessary to prove the efficacy of compression, but a continuous monitoring doesn't seem necessary; c) in order to stop the procedure perhaps it is sufficient to interrupt completely the flow in the pseudoaneurysm cavity; a small flow, limited to the neck of the lesion, can be resolved if a pressure bandage is applied and the patient is maintained at bed rest for 12 hours. Manual, ultrasound guided obliteration may be considered a safe and efficacious method for non-surgical treatment of femoral artery pseudoaneurysms.

[Involvement of the heart valves and great vessels in homozygote familial hypercholesterolemia]. / Interessamento delle valvole cardiache e delle grandi arterie nella ipercolesterolemia familiare omozigote.

Homozygous familial hypercholesterolaemia is characterized by cutaneous xanthoma development from infancy, precocious and accelerated atherosclerosis with clinical signs of ischemic heart disease and frequent involvement of left heart valves resulting in stenosis and/or incompetence. Two cases are described of this condition, both associated with aortic stenosis. In one case mitral incompetence and thromboembolic pulmonary hypertension were also found. The mitral valve is involved in the atherosclerotic process at the level of the cusps. These become thickened and stiff. Aortic stenosis is mainly due to atheromas infiltrating the Valsalva sinuses and the ascending aorta. Pulmonary hypertension, never reported before in this disease, is probably due to concomitant atheromatosis involving the pulmonary artery with secondary fatty embolism.

[Mitral valve replacement in young children: a long-term follow-up]. / Sostituzione valvolare mitralica nella prima infanzia: follow-up a lungo termine.

The Authors report follow-up data (mean 60, range 13-101 months) from 4 patients under two years of age (mean 11.5 months) operated on for prosthetic mitral valve implantation. Two patients were male and two female. Pre-operative diagnosis was partial atrioventricular septal defect with parachute mitral valve in one case and isolated mitral valve anomaly in the others (1 parachute, 2 hammock valves). One bioprosthesis (Liotta n. 23) and three mechanical prostheses (Björk-Shiley) were implanted. Complications occurred in two patients: one case of endocarditis on the bioprosthesis, leading to substitution with a Björk-Shiley valve; one case of acute thrombosis 8 months after implantation in the only pt receiving aspirin as prophylactic therapy. This complication was resolved by intravenous thrombolytic agents. All the patients are alive, in I NYHA functional class and receiving oral anti-coagulants. Instrumental follow-up with eco-Doppler demonstrated normal functioning prostheses in all the patients. A sub-aortic gradient was found in the patient with partial atrioventricular septal defect. Prosthesis-patient mismatch was identified in a patient operated on when she was 6 months old who was followed-up for 5 years.

[Pulmonary arteriovenous aneurysm coexisting with rheumatic mitral valvulopathy in Rendu-Osler-Weber syndrome. Image diagnosis using bidimensional and Doppler echocardiography]. / Aneurisma arterovenoso polmonare coesistente con valvulopatia mitralica reumatica in sindrome di Rendu-Osler-Weber. Diagnostica per immagini con ecocardiografia bidimensionale e Doppler.

The authors report a case of the rare association described in the title in which echocardiography played an important role in the recognition of the pulmonary arteriovenous aneurysm. By 2-D approach it was possible to visualize a wide anechogenic concamerated area containing an echodense mass and continuous with the left heart cavities. Pulsed Doppler interrogation at the level of the extra-cardiac cavity showed a flow signal which gave the first diagnostic evidence of a vascular lesion. Direct visualization of pulmonary arteriovenous aneurysms has never before been obtained with echocardiography. This was possible in the present case due to contiguity with heart chambers and the large dimension of the aneurysm. Doppler examination gave the first confirmation of the vascular nature of the lesion. Angiography is still a mandatory pre-operative procedure but 2-D and Doppler echocardiography may play an interesting role in the diagnostic non invasive procedure of pulmonary arteriovenous aneurysms.

[Atresia of the tricuspid valve and total anomalous supracardiac pulmonary venous return. Description of a case associated with severe extracardiac malformations]. / Atresia della tricuspide e ritorno venoso polmonare anomalo totale sopracardiaco. Descrizione di un caso associato a gravi malformazioni extracardiache.

The Authors report a case of tricuspid atresia and total anomalous pulmonary venous return of the supracardiac type, with mild pulmonary valve stenosis and extracardiac malformations as anal atresia, microphthalmia and irideo and chorioretinic coloboma. The Authors stress the importance of the electrocardiogram and standard chest X-Ray to "suspect" this rare association.

[Angiocardiography with axial projections in the study of various types of intraventricular septal defects]. / L'angiocardiografia con proiezioni assiali nello studio dei vari tipi di difetto del setto interventricolare.

The exact site of an interventricular septal defect (VSD) is important mainly to program surgical treatment. The present study takes into consideration the possibility of the axial angiocardiographic method to identify the site of the interventricular septal defect. With this goal 126 cases of VSD in infancy were retrospectively studied with left ventriculograms using angled "long-axial" and "four chambers" views. In 62 cases the anatomic-angiographic correlation of the VSD site was reached, and was confirmed at surgery. The most frequent localization of the VSD was perimembranous (89 cases, 70%). The rarest localizations were the sub-arterial and the multiple VSD. The personal experience agrees with the data found in literature, namely that angiocardiography performed with axial views has an elevated diagnostic reliability in the various types of VSD.

[Clinical and angiocardiographic observations on the so-called absence of a branch of the pulmonary artery]. / Osservazioni cliniche ed angiocardiografiche sulla cosiddetta "assenza" di un ramo dell'arteria polmonare.

We present 5 cases with angiographically absent pulmonary artery: 4 with absence of right pulmonary artery and 1 with absence of the left. We excluded cases of atresia of pulmonary artery, ventricular septal defect and major aorto-pulmonary collaterals. We excluded also cases of anomalous origin of a pulmonary artery from the aorta. Surgical inspection in 3 patients demonstrated total absence of pulmonary main branch (1 case), with intraoperative death, and presence of the hilar portion of the pulmonary artery (2 cases), with successful palliative procedure. It is supportive of efforts to identify as early in life as is feasible the absent vessel, for surgical purposes. In fact the early surgical procedure will ensure development of the vessel and normal lung growth, and will decrease the risk of the following intracardiac repair, when needful.

Broncho-atrial discordance. A clinically diagnosed case.

Determination of the arrangement of the atrial chambers ("atrial situs") is the first step in sequential chamber analysis of complex congenital heart disease. Although bronchial pattern, as detected by chest X-ray, is an excellent guide for prediction of atrial arrangement, exceptions to this rule based on post-mortem examination have been described. We report a case in which discordance between the atrial arrangement (mirror-image) and bronchial morphology (usual) was diagnosed during life.

Supraventricular tachycardia in infants: use of the "diving reflex".

The effectiveness of the "diving reflex" in treating paroxysmal supraventricular tachycardia (PSVT) in 10 infants aged under 6 months is demonstrated. No rhythm disturbances were encountered during the maneuver, even in patients receiving digitalis. The recommended water temperature is 5 degrees C and the total time of facial immersion, between 6 and 7 seconds. The diving reflex might be considered the first therapeutic approach in infants under 6 months old with PSVT.

[Scimitar syndrome with or without the "scimitar sign"]. / Sindrome della scimitarra con o senza il "segno della scimitarra".

The so called scimitar syndrome is a congenital malformation characterized mainly by the anomalous venous return of the right lung in inferior vena cava. Many structure of the lung may be involved. Usually lobation and bronchial distribution are abnormal, the right pulmonary artery is hypoplastic or absent, a partial anomalous artery from aorta (pulmonary sequestration) may be present, the entire lung is small and various degree of displacement of the heart to the right is detectable. Associated congenital heart defects may also be present. The name of the syndrome comes from the presence of this vessel (scimitar's sign) on routine chest x-ray. This paper describes the clinical and angiographic reports of four cases, which may represent, according a unique embriological interpretation, a different instances of this syndrome. The anatomic characteristic, the sign of scimitar, could be also absent, as in cases 2 and 3. In the case 2 there was complete absence of right pulmonary artery and in the case 3 a severe hypoplastic right pulmonary artery with unique small venous return in left atrium. The authors opinion is that all the anomalies of lung vascular connections, that result from interference with the normal growth of the lung bud, its separation from systemic vascular channels, and the establishment of the lesser circulation should be included. Because the different components of thie malformation can be present in various combination, the sign of scimitar is not pathognomonic to define the syndrome.

[Persistence of fetal circulation. Clinical and angiographic evaluation of two cases. Review of the literature (author's transl)]. / Persistenza della circolazione fetale. Descrizione clinica ed angiocardiografica di due casi. Revisione della letteratura.

Persistent fetal circulation ("PFC") is a condition characterized by perduring after birth of fetal hemodynamic pattern: persistence of high pulmonary vascular resistance then occurs. "PFC" is a high mortality syndrome recently recognized as a cause of severe cyanosis and tachypnea in full-term newborns. Perinatal hypoxia, hyperviscosity, acidosis etc, may be also responsible of a persistence of high pulmonary vascular resistance. The increased right heart pressures cause a right to left shunt across the ductus arteriosus and foramen ovale not yet completely closed at birth. In this paper the clinical and angiographic evaluation of two full-term newborns, males, 5 days and 5 months old, are described, who presented with tachypnea, cyanosis and heart failure. Moreover we want to make a critical review of medical literature about this subject. The electrocardiogram of both patients showed right ventricular hypertrophy and myocardial ischemia. The chest roentgenograms showed cardiac enlargement and increased pulmonary vascular markings. Lung and central nervous system disorders were excluded by clinical and instrumental exams. Both patients underwent cardiac catheterization and angiocardiography in order to exclude a congenital heart disease. There was just evidence of pulmonary vascular resistance and right to left shunt at ductal and atrial level. O2 100% administration in one patient determined a decrease in pulmonary resistance and the shunting abolition. Clinical and hemodynamic patterns of this syndrome are very different. There are "simple forms" with normal myocardial function and "complex" higher mortality forms with evidence of cardiac involvement. It is often difficult to distinguish such forms from the other causes of central cyanosis and/or cardiac failure in the newborn as transposition of the great arteries, total anomalous pulmonary venous connection: the individualization of the syndrome then may be impossible without cardiac catheterization. A correct diagnosis for a precocious and reasonable treatment are extremely important for the survival of such critical newborns.

[Interventricular defect with discrete aortic stenosis below the defect]. / Difetto interventricolare con stenosi sottoaortica discreta sotto il difetto.

"Discrete" (fixed) subaortic stenosis associated with ventricular septal defect (VSD) is a rare but important anomaly. Two types of left ventricular outflow tract obstruction should be distinguished on the basis of its relation with the VSD, depending on whether the stenosis is above or below the defect. The four cases presented here are all in the latter category. Usually the patients belonging in the former category are associated with severe anomalies of the aortic arch. The diagnosis was made by clinical examination and by means of non invasive techniques, ecg, chest x-rays and ecocardiograms in all but one patient (the case "3"), in whom a subpulmonary stenosis (SPS) was associated. The final diagnosis was established by catheterization, which demonstrated the pressure gradient on the withdrawal curve from the apical part of the left ventricle to the aorta, and by contrastography. Three of four patients underwent total correction and are in good condition from 6 to 18 months after surgery, one of these cases had also SPS. In the remaining case, we thought the operation was not indicated on the basis of small size of VSD, of mild pressure gradient, and mild aortic valvular insufficiency (case "4"). The surgical approach to resect the "fixed" obstruction and to closure the VSD was carried out through a right atriotomy in two patients, and through a right ventriculotomy in the patient with SPS. The trans-aortic approach has to be discarded because it affords limited exposure of both defects and could increase the risk of damage of conducting tissue. A careful evaluation of aortic and left ventricular pressure, in association with angled angiography is highly recommended in the study of VSD.

[Azygos or hemiazygos continuation of the inferior vena cava. An angiographic findings of 10 cases (author's transl)]. / Continuazione azigos o emiazigos della vena cava inferiore. Studio angiografico di 10 casi.

The angiographic findings of 10 cases with azygos (AC) or hemiazygos (HC) continuation of the inferior vena cava (IVC), associated with other congenital heart disease (0.5%), are described. Abdominal situs solitus was present in 7 cases, left isomerism in 3. In 5 cases IVC was to the left to the spine, bilateral in one. In 6 cases there was AC, HC in 2; in one both types of continuation were documented. It is important that this venous anomaly be diagnosed before cardiac catheterization; manipulation of the probe may be very difficult when saphenous approach is used. The correct use of the terms "Azygos", "Hemiazygos" and "Accessory Hemiazygos" are discussed.