scholarly journals Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report

2017 ◽  
Vol 5 (1) ◽  
pp. 53-56
Author(s):  
Rahul Regi Abraham ◽  
Rahul Regi Abraham
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Pulmonary Stenosis ◽  
Eisenmenger Syndrome ◽  
Tertiary Center ◽  
History Of ◽  
Double Inlet Left Ventricle

Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.

scholarly journals Double Inlet Left Ventricle (DILV), Double Outlet Left Ventricle (DOLV), Malposition of Great Arteries, Inlet Ventricle Septal Defect (VSD) and Patent Ductus Arteriosus

2016 ◽  
pp. 157-61
Author(s):  
Sylvie Sakasasmita ◽  
Ganesja M Harimurti ◽  
Dicky Fakhri ◽  
Venty Venty
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Patent Ductus Arteriosus ◽  
Congenital Heart ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Fontan Procedure ◽  
Double Inlet Left Ventricle ◽  
Patent Ductus

Congenital heart disease is an interesting area which present a great various arrangement of the cardiac stucture. We present a rare case of 11 months old male with complex congenital heart disease consisted of Double Inlet Left Ventricle (DILV), Double Outlet Left Ventricle (DOLV) with Malposition of Great Arteries, Inlet Ventricle Septal Defect (VSD) and Patent Ductus Arteriosus (PDA).Patient was an eleven months old male who was admitted in our institution for cardiac operation. He was diagnosed with congenital heart disease since three days old with initial presentation of cyanosis when he was crying. His physical growth was retarded but his developmental Milestones was considered normal. On admission, his oxygen saturation was 88% with ambient air. The diagnosis was confirmed by echocardiography. He was planned to undergo staging surgery which would end to Fontan Procedure. Pulmonary Artery (PA) banding was performed to reduce blood flow to pulmonary circulation, distribute more blood from the left ventricle to aorta and systemic circulation and prepare for bidirectional Glenn Shunt procedure one year later and Fontan procedure a year after that.

scholarly journals Double Inlet Left Ventricle (DILV), Double Outlet Left Ventricle (DOLV), Malposition of Great Arteries, Inlet Ventricle Septal Defect (VSD) and Patent Ductus Arteriosus

2017 ◽  
pp. 28-32
Author(s):  
Sylvie Sakasasmita ◽  
Ganesja M. Harimurti ◽  
Dicky Fakhri ◽  
Venty Venty
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Patent Ductus Arteriosus ◽  
Congenital Heart ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Fontan Procedure ◽  
Double Inlet Left Ventricle ◽  
Patent Ductus

Congenital heart disease is an interesting area which present a great various arrangement of the cardiac stucture. We present a rare case of 11 months old male with complex congenital heart disease consisted of Double Inlet Left Ventricle (DILV), Double Outlet Left Ventricle (DOLV) with Malposition of Great Arteries, Inlet Ventricle Septal Defect (VSD) and Patent Ductus Arteriosus (PDA).Patient was an eleven months old male who was admitted in our institution for cardiac operation. He was diagnosed with congenital heart disease since three days old with initial presentation of cyanosis when he was crying. His physical growth was retarded but his developmental Milestones was considered normal. On admission, his oxygen saturation was 88% with ambient air. The diagnosis was confirmed by echocardiography. He was planned to undergo staging surgery which would end to Fontan Procedure. Pulmonary Artery (PA) banding was performed to reduce blood flow to pulmonary circulation, distribute more blood from the left ventricle to aorta and systemic circulation and prepare for bidirectional Glenn Shunt procedure one year later and Fontan procedure a year after that.

Double inlet left ventricle associated with a subpulmonary tissue tag, dissection of the pulmonary trunk and pheochromocytoma

1997 ◽  
Vol 7 (3) ◽  
pp. 334-336 ◽  
Author(s):  
Shaun A.C. Medlicott ◽  
Joyce R. Harder ◽  
Lloyd N. Denmark
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Pulmonary Trunk ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
The Individual ◽  
Double Inlet Left Ventricle

AbstractPrevious studies have demonstrated the individual association of subpulmonary tissue tags, dissection of the pulmonary trunk and primitive neuroectodermal tumors with congenital heart disease. We are reporting a case of double inlet left ventricle with all these associations as it is exceptionally rare for all features to be manifest in one individual.

scholarly journals Double Inlet Left Ventricle (DILV) with Mal-posed Great Vessels: A Challenge in a Developing World

2021 ◽  
pp. 20-24
Author(s):  
Josephat M. Chinawa ◽  
Awoere T. Chinawa ◽  
Chika O. Duru ◽  
Ann E. Aronu
Keyword(s):  
Congenital Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Surgical Intervention ◽  
Left Ventricular ◽  
Cardiac Anomaly ◽  
Great Vessels ◽  
History Of ◽  
High Index Of Suspicion ◽  
Double Inlet Left Ventricle

Background: Double Inlet Left Ventricle (DILV) with transposition of the great vessels is rare in children with congenital heart disease. We present two cases seen at a paediatric echocardiography centre. Baby ND is a 28-day old female who presented with respiratory distress and cyanosis from the first day of life. Echocardiography showed a double inlet left ventricle and mal-posed great vessels. MB is a seven-month-old female who presented with breathlessness and cyanosis from the first day of life. Examination showed a soft pan-systolic murmur of grade 2 variety at the mid sternal boarder. ECG showed left ventricular dominance and abnormal T changes. Echocardiography revealed a double inlet left ventricle and mal-posed great vessels.  Conclusion: Double inlet left ventricle and mal-posed great vessels is a rare complex cardiac  anomaly of univentricular physiology. A high index of suspicion (especially if the new born presents with first day history of cyanosis and breathlessness) is necessary for quick referral and surgical intervention.

Reversing Inoperability in Eisenmenger Syndrome: The “Drug-and-Banding” Approach

2020 ◽  
Vol 11 (5) ◽  
pp. 646-648
Author(s):  
Nicola Pradegan ◽  
Rita Pesce ◽  
Biagio Castaldi ◽  
Lorenza Zanotto ◽  
Giovanni Stellin ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ventricular Septal Defect ◽  
Pulmonary Vascular Resistance ◽  
Congenital Heart ◽  
Septal Defect ◽  
Functional Improvement ◽  
Eisenmenger Syndrome ◽  
Vasodilator Therapy

Eisenmenger syndrome (ES) has been considered a cause of inoperability in patients with congenital heart disease (CHD). Even if long-standing vasodilators are efficient to reduce pulmonary vascular resistance (PVR), the best approach to grant stable repair of these patients is still unknown. We describe the case of a 28-year-old man with a diagnosis of untreated large ventricular septal defect and established ES. After few years of vasodilator therapy, the patient underwent pulmonary banding with significant reduction of his PVR. His CHD was then repaired, with clinical and functional improvement at 2-year follow-up.

Risk factors for loss to follow-up among children and young adults with congenital heart disease

2011 ◽  
Vol 22 (3) ◽  
pp. 307-315 ◽  
Author(s):  
Andrew S. Mackie ◽  
Gwen R. Rempel ◽  
Kathryn N. Rankin ◽  
David Nicholas ◽  
Joyce Magill-Evans
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Confidence Interval ◽  
Odds Ratio ◽  
Congenital Heart ◽  
Family Income ◽  
Loss To Follow Up ◽  
History Of

AbstractObjectiveTo identify risk factors for loss to cardiology follow-up among children and young adults with congenital heart disease.MethodsWe used a matched case-control design. Cases were born before January, 2001 with moderate or complex congenital heart disease and were previously followed up in the paediatric or adult cardiology clinic, but not seen for 3 years or longer. Controls had been seen within 3 years. Controls were matched 3:1 to cases by year of birth and congenital heart disease lesion. Medical records were reviewed for potential risk factors for loss to follow-up. A subset of cases and controls participated in recorded telephone interviews.ResultsA total of 74 cases (66% male) were compared with 222 controls (61% male). A history of missed cardiology appointments was predictive of loss to follow-up for 3 years or longer (odds ratio 13.0, 95% confidence interval 3.3–51.7). Variables protective from loss to follow-up were higher family income (odds ratio 0.87 per $10,000 increase, 0.77–0.98), cardiac catheterisation within 5 years (odds ratio 0.2, 95% confidence interval 0.1–0.6), and chart documentation of the need for cardiology follow-up (odds ratio 0.4, 95% confidence interval 0.2–0.8). Cases lacked awareness of the importance of follow-up and identified primary care physicians as their primary source of information about the heart, rather than cardiologists. Unlike cases, controls had methods to remember appointments.ConclusionsA history of one or more missed cardiology appointments predicted loss to follow-up for 3 or more years, as did lack of awareness of the need for follow-up. Higher family income, recent catheterisations, and medical record documentation of the need for follow-up were protective.

Sign in / Sign up

Export Citation Format

Share Document