Cyanotic Congenital Disease Research Articles

Profile of hypospadias patients at Dr. Moewardi General Hospital Surakarta period 2015-2020

Background: Hypospadias is the second most common congenital malformation in males and the most common congenital malformation of the penis. This malformation is characterized by the anatomical position of the urinary tract opening on the ventral or anterior part of the penis. The prevalence of hypospadias in western countries around 8 of 1000 live births and is reported to increase every year.Methods: This study is a descriptive study with a retrospective cross-sectional approach. All data were retrieved from RSUD Dr. Moewardi Surakarta using hypospadias patients medical records from January 2015 – December 2020. The hypospadias case were classified into mild, moderate, and severe hypospadia based on location. The age of being diagnosed, concomitant congenital disease, surgery technique, and surgical complication were described.Results: There were 120 cases of hypospadias, consisted of 47.5% severe hypospadias, 34.9% moderate hypospadias and 17.4% mild hypospadias. Patients’ age of being diagnosed was most in the 1-5 years group (35.9%). Seventy-eight (75.7%) cases without concomitant congenital disease, 10.7% with undecensus testiculorum, 4.8% with cyanotic congenital disease, and 3.8% with cloacal. There were 68.9% cases repaired with first-stage surgical technique and 31.1% repaired with two-stage surgical technique. Most surgical complications were meatal stenosis 9%.Conclusions: Majority of hypospadias cases were severe hypospadia. Other profile like age of being diagnosed, concomitant congenital disease, surgery technique, and surgical complication were found in this study.

Transcatheter Floating Valve Syndrome in Recurrent Congenital Pulmonary Valve Stenosis

Objective: Complications of percutaneous pulmonary valve implantation (PPVI) remain underreported. We report treatment of recurrent right ventricular outflow tract (RVOT) obstruction and discuss management of a dislodged valve during PPVI. Methods: A 36-year-old female with Tetralogy of Fallot and five previous sternotomies presented with progressive dyspnea. TEE revealed preserved ejection fraction (50-55%), severe pulmonic stenosis (mean gradient: 26-mmHg; peak gradient: 60-mmHg), and regurgitation. She underwent PPVI using a 23-mm Sapien valve. Initial deployment was unsuccessful, resulting in a free-floating valve in the right ventricle (RV). A second oversized percutaneous valve was positioned more distally, deployed with extra balloon dilatation. Second deployment was successful with RVOT gradient relief. The floating valve was then addressed. Two techniques were attempted: “jailing” and “snare-and-crush” (Figure 1A and 1B). Although snaring was successful and the valve pulled into the inlet of the RV, crushing proved difficult. No further attempt was made due to possible tricuspid valve damage. The patient was converted to a fourth-time redo-sternotomy with retrieval of the valve (Figure 1C). Her post-op course was uneventful. Results: Detachment of the initial valve resulted from landing zone miscalculation. In second valve placement, more distal deployment and sizing to a 29-mm valve allowed for better stabilization. Although the accordion-style metal frame of the valve lends itself to a snare-and-crush approach, this proves difficult in practice. Another impediment to successful percutaneous retrieval lies in the complex tricuspid sub-valvar apparatus and potential iatrogenic injury. Conclusions: PPVI is important in managing recurrent cyanotic congenital disease. Reporting of complications is important to minimize risk and understand management strategies. We report on careful assessment of the landing zone and discuss useful strategies to manage valve dislodgement.

Transcatheter, Occlusion Of Patent Ductus Arteriosis Using Rashkind’s Umbrella Device

Summary: Transcatheter occlusion of PDA using Rashkind’s Umbrella device is a popular method of PDA closure in the first world today. We performed our first successful closure on 20.9.1994 at AFIC/NIHD, Rawalpindi. Discussion: Ductus arteriosis is an aiterial connection between the descending aorta (just distal to the origin of the left subclavian artery) and the left pulmonary artery close to its origin. It closes within few hours to few weeks from birth. It causes increased left to right shunt and predisposes to volume overload of the heart, infections, infective endocarditis and to calcification, aneurysm formation and even rupture at late stages. Except when PDA is part of congenital cyanotic heart disease and helping the duct dependent circulation, the PDA has to be closed, and depending on clinical urgency various methods are employed such as intrav enous Indomethacin, or surgical intervention like ligation or duct interruption.

Acute symptomatic seizures as a complication of rhinosinusitis in children

When an otorhinolaryngologist thinks of acute seizures in children, what comes to mind are intracranial complications of infection within the sinuses or the temporal bone. Complications of rhinosinusitis, both in children and adults, may involve the orbit, soft tissues surrounding the nose and sinuses, and intracranial spread of the infection. Intracranial complications secondary to rhinosinusitis, intracranial abscesses and/or thrombophlebitis, occur sporadically and, although it appears that they cannot be prevented, early recognition and treatment are essential to reduce any subsequent morbidity or mortality [1]. The commonest presenting symptoms of intracranial complications are headaches (80%) followed by vomiting (45%) and swelling of the forehead (45%), an altered mental state, fever, seizure, unilateral weakness or hemiparesis, or a cranial nerve sign [1,2]. Exact pathogenesis of intracranial abscesses remains unclear [3]. Apart from rhinosinusitis, congenital cyanotic heart disease was identified as a predisposing factor, likewise otitis media or mastoiditis, dental abscesses and immunodeficiency [3]. The diagnosis of cerebral abscess is often difficult. The classical intracranial hypertension associated to high fever is usually incomplete and sometimes absent [4]. In most cases (60%), it is from the frontal sinus that the infection spreads intracranially [2]. In patients with intracranial complications of rhinosinusitis, frequency of seizures reaches 10–25% [1,3,5]. Epilepsy caused by intracranial complications is usually a long-term morbidity [1]. Above enumerated presenting symptoms justify an urgent magnetic resonance imaging (MRI) or computed tomography (CT) scan. The importance of imaging before a lumbar puncture cannot be overemphasized [1,6]. Since the availability of CT, both diagnostic delay after hospital admission and mortality were substantially reduced: mean delay from 8 to 3 days, andmortality from 25% to almost 0% [3]. In patients with intracranial complications, acute symptomatic seizures are usually accompanied by other neurological symptoms: fever in 50% of cases, headache in 75% of cases, and intracranial hypertension syndrome in 25% of cases [4]. The condition requires sinus surgery in order to drain the primary focus of the infection [2,7]. At the same time, prompt and aggressive medical and neurosurgical intervention is required, aiming tominimize the morbidity and mortality and to maximize the favorable outcome in those children [2]. The neurosurgical procedures aim to drain the abscess and include burr whole drainage or aspiration of abscess, craniotomy and evacuation of empyema, or craniotomy [2]. Central nervous system scars with subsequent seizures can be sequels of intracranial abscess therapy and are more common after excision of the brain abscess when compared to patients treated by aspiration and/or antibiotics alone [2,3,7]. Therefore, excision of brain abscess should be avoided *Address for correspondence: Olaf Zagolski, ENT, St. John Grande’s Hospital, Krakow, Poland. Tel.: +48 12 3797377; Fax: +48 12 4305491; E-mail: olafzag@poczta.onet.pl. Journal of Pediatric Epilepsy 1 (2012) 73–74 DOI 10.3233/PEP-2012-012 IOS Press 73

The Role Of Endothelial Nitric Oxide Synthase Expression In The Development Of Pulmonary Hypertension In Chronically Hypoxic Infant Swine

Objective: Our goal was to determine the role of pulmonary endothelial nitric oxide synthase expression in the development of pulmonary hypertension in infants with congenital cyanotic heart disease. Methods: Two groups of 4-week-old piglets were studied. In one group, the piglets were raised in an environment of 10% oxygen from 2 days of age (cyanotic, n = 6), and in the other group the piglets were raised at room air (control, n = 5). Pulmonary hemodynamics were measured in vivo for each animal, and peripheral lung biopsy specimens were obtained for Western blot analysis with the use of antiendothelial nitric oxide synthase antibody and for activity analysis with the use of the tritiated l-arginine assay. Results: The piglets in the chronically hypoxic group had significant increases in mean pulmonary arterial pressure (44.0 ± 3.8 mm Hg vs 14.8 ± 1.2 mm Hg in controls, p = 0.0007) and pulmonary vascular resistance (7272.0 ± 871.1 dyne · cm · sec -5 vs 1844.5 ± 271.2 dyne · cm · sec -5in controls, p = 0.002). These changes in the pulmonary hemodynamics of the hypoxic piglets were accompanied by a twofold increase in the expression of pulmonary endothelial nitric oxide synthase ( p = 0.0043) but no corresponding increase in nitric oxide synthase activity. Conclusions: Raising infant piglets in an environment of 10% oxygen for 4 weeks results in significant pulmonary arterial hypertension accompanied by increased expression of nitric oxide synthase within the lung endothelium. Furthermore, the increased levels of nitric oxide synthase within the lungs of the hypoxic swine were not accompanied by a proportional increase in enzyme activity. These findings suggest that the development of pulmonary hypertension in infants with congenital cyanotic disease is not due to decreased expression of endothelial nitric oxide synthase, but instead may be related to a decreased ability of the enzyme to produce sufficient nitric oxide. (J Thorac Cardiovasc Surg 1998;115:343-50)

Platelet kinetics in congenital heart disease.

The survival of 111indium labelled platelets has been determined in a series of 47 subjects comprising nine with cyanotic congenital disease (Eisenmenger's syndrome), seven with congenital heart disease associated with left to right shunts, six with primary pulmonary hypertension, six with peripheral vascular disease, 11 with cardiac disorder associated with low cardiac output and eight normal volunteers. Compared with the value in the normals of 9.5 days, mean survival was significantly shortened in those with Eisenmenger's syndrome (8.4 days) and with peripheral vascular disease (8.5 days). It was normal in patients with left to right shunts (9.5 days). Gamma camera imaging in selected patients failed to reveal any abnormal sites of deposition of labelled platelets except in one patient with peripheral vascular disease who had bilateral abnormal activity in his lower limbs and a shortened platelet survival (8.0 days). From theoretical considerations, it was concluded that the reduction in platelet survival in Eisenmenger's syndrome was such that, had it been the result of pulmonary intravascular platelet deposition, abnormal activity should have been visible on chest scanning with the gamma camera. The absence of scintigraphic evidence of abnormal platelet deposition in the lungs of these patients, combined with the linear configuration of their platelet survival curves, suggests that the accelerated platelet destruction is in the reticuloendothelial (RE) system rather than intravascular. Indirect evidence in favour of increased RE destruction of platelets in Eisenmenger's syndrome was the finding of an approximate doubling of intrasplenic platelet transit time, indicating abnormal platelet pooling within the spleen.

Wire coil embolization of systemic-pulmonary artery collaterals following surgical correction of pulmonary atresia

This report describes the selective obliteration of large systemic-pulmonary arterial collateral vessels, commonly found in cyanotic congenital disease with severe pulmonary atresia. A transcatheter wire coil embolus technique was used. The relative ease with which these shunt vessels can be occluded, with concomitant decrease in morbidity, is stressed and the importance of a team approach in the perioperative evaluation, angiographic assessment, and eventual therapy of patients with these complex anomalies is emphasized.

The disappearance of foetal haemoglobin in congenital cyanotic heart disease.

The disappearance of foetal haemoglobin in congenital cyanotic heart disease.