The more severe the obstruction, the earlier it may present. On the other hand, those with milder obstruction may not present until late adulthood. [achaheart.org]

[citation needed] Coarctation of the aorta which is, narrowing of a section of the aorta may also be observed.[1] Again this presents an obstruction to blood flow out from the left ventricle. [en.wikipedia.org]

In the complete form, all of the mentioned defects are present. Partial complexes have also been described in which some of the obstructive lesions are present but others are absent or clinically nonsignificant. [brieflands.com]

Generally, patients will need multiple surgeries at initial presentation, as well as repeated surgeries throughout their life 2,3. [radiopaedia.org]

True Shone’s complex, when all four defects are present, is rare and the term Shone's like complex is often used to refer to multiple sites of left sided obstruction and hypoplasia. References: Lai, W. (2009). [pedecho.org]

• Coarctation of the Aorta

  Coarctation of the Aorta. Coarctation of the aorta is a narrowing of the aorta at the level of the ductus arteriosus. The ductus arteriosus is a blood vessel found in the fetus that allows blood to bypass the lungs. [achaheart.org]

  It is a series of four obstructive or potentially obstructive left-sided cardiac lesions consisting of supravalvular mitral ring, parachute deformity of the mitral valve, subaortic stenosis, and coarctation of the aorta. [accessanesthesiology.mhmedical.com]

  […] of the aorta. [pedecho.org]

  Coarctation of the aorta – a narrowing or constriction of the aorta, the large vessel that carries blood from the heart to the body tissues. 2. [pted.org]

  Its complete form associates a supra-mitral valve ring, sub aortic stenosis, parachute mitral valve (PMV), and coarctation of the aorta. [ncbi.nlm.nih.gov]

• Fatigue

  Symptoms of congestive heart failure (which can occur in the first week of life) include fatigue, rapid breathing and wheezing, faster than normal heart rate, poor oral intake, poor weight gain, fluid retention (edema) in the legs, pallor (anemia), and [nicklauschildrens.org]

  As the child grows, they may have symptoms like fatigue, coughing at night, and wheezing. [myacare.com]

  The children tend to show symptoms like fatigue, nocturnal cough, and reduced cardiac output by the age of two years. They also develop wheezing due to the exudation of fluid into the lungs. [en.wikipedia.org]

  Fatigue or extreme sleepiness, such as becoming too tired to eat. Sweating. What causes Shone’s complex? Healthcare providers don’t always know exactly what causes Shone’s complex. [my.clevelandclinic.org]

  Other surgical procedures depend on the existence of associated cardiac anomalies, which can determine late surgical outcome.[2],[9] The usual presentation is dyspnea, nocturnal cough, tachypnea, poor feeding, failure to thrive, fatigue, signs, and symptoms [rcvmonline.com]

• Atrial Septal Defect

  The most frequent associated conditions were atrial septal defect 34%, ventricular septal defect 29% and left superior vena cava 14%. Progression was found in 55% of the patients. [ahajournals.org]

  septal defect, ventricular septal defect). [pted.org]

  Associated congenital defects such as VSD, PDA, and atrial septal defect maybe seen specially in the partial form (8, 9). [brieflands.com]

• Congestive Heart Failure

  Symptoms of congestive heart failure (which can occur in the first week of life) include fatigue, rapid breathing and wheezing, faster than normal heart rate, poor oral intake, poor weight gain, fluid retention (edema) in the legs, pallor (anemia), and [nicklauschildrens.org]

  Babies born with Shone’s complex may also have symptoms of congestive heart failure, such as: Cough and congestion in the lungs. Difficulty breathing. Fatigue or extreme sleepiness, such as becoming too tired to eat. Sweating. [my.clevelandclinic.org]

• Turkish

  Bezmialem Vakıf Üniversitesi Tıp Fakültesi, Kardiyoloji Bölümü, İstanbul Archives of the Turkish Society of Cardiology 2012; 40: 350-353 DOI: 10.5543/tkda.2012.27003 Read: 433 Downloads: 140 Published: 01 July 2021 Volume 40, Issue 4, June 2012 Shone’ [archivestsc.com]

• Cough

  As the child grows, they may have symptoms like fatigue, coughing at night, and wheezing. [myacare.com]

  The children tend to show symptoms like fatigue, nocturnal cough, and reduced cardiac output by the age of two years. They also develop wheezing due to the exudation of fluid into the lungs. [en.wikipedia.org]

  Babies born with Shone’s complex may also have symptoms of congestive heart failure, such as: Cough and congestion in the lungs. Difficulty breathing. Fatigue or extreme sleepiness, such as becoming too tired to eat. Sweating. [my.clevelandclinic.org]

  Other surgical procedures depend on the existence of associated cardiac anomalies, which can determine late surgical outcome.[2],[9] The usual presentation is dyspnea, nocturnal cough, tachypnea, poor feeding, failure to thrive, fatigue, signs, and symptoms [rcvmonline.com]

• Dyspnea

  During the two year follow-up period, in spite of an atrial fibrillation attack and deteriorating dyspnea, the patient still did not want another operation. [archivestsc.com]

  Shortness of breath (dyspnea), especially around feeding time. Babies born with Shone’s complex may also have symptoms of congestive heart failure, such as: Cough and congestion in the lungs. Difficulty breathing. [my.clevelandclinic.org]

  The child had been regularly followed thereafter until he became symptomatic at 3 years of age with dyspnea on exertion (DOE). [brieflands.com]

  Other surgical procedures depend on the existence of associated cardiac anomalies, which can determine late surgical outcome.[2],[9] The usual presentation is dyspnea, nocturnal cough, tachypnea, poor feeding, failure to thrive, fatigue, signs, and symptoms [rcvmonline.com]

• Heart Disease

  Quiz questions References Related articles: Pathology: Vascular: Congenital heart disease Promoted articles (advertising) [radiopaedia.org]

  Incidence Occurs in 0.6% of children with congenital heart disease. There is no racial or sex predilection. Clinical aspects This syndrome is present at birth. [accessanesthesiology.mhmedical.com]

  Shone’s complex is a congenital (present at birth) heart disease. It affects how blood flows both into and out of the left side of the heart. [my.clevelandclinic.org]

  Shone’s syndrome, is a rare form of congenital heart disease where there is a combination of four left-sided heart defects (obstructions), which include: Aortic coarctation (narrowing of the aorta) Obstruction below the aortic valve (subaortic obstruction [nicklauschildrens.org]

  Echocardiography in Pediatric and Congenital Heart Disease. West Sussex, UK: Wiley-Blackwell. Reynolds, Terry. (2002). The Pediatric Echocardiographer’s Pocket Reference (3rd ed.). Arizona: Arizona Heart Institute [pedecho.org]

• Hypertension

  References Combined atrial arterial switch operation (double switch) for hearts with Shone syndrome and pulmonary hypertension [bchcicu.org]

  Undilated right ventricle with, good systolic function, No sign of pulmonary hypertension. [ncbi.nlm.nih.gov]

  This can causes fl uid to back up into the lungs (pulmonary edema), pulmonary arterial hypertension (high blood pressure in the lungs), and fast heart beats (arrhythmias). [achaheart.org]

  [citation needed] Treatment[edit] When treated early, that is, before the onset of pulmonary hypertension, a good outcome is possible in patients with Shone’s syndrome. [en.wikipedia.org]

  He also suffered from hypertension in the past year. [rcvmonline.com]

• Heart Failure

  Symptoms of congestive heart failure (which can occur in the first week of life) include fatigue, rapid breathing and wheezing, faster than normal heart rate, poor oral intake, poor weight gain, fluid retention (edema) in the legs, pallor (anemia), and [nicklauschildrens.org]

  A note from Cleveland Clinic Shone’s complex is a congenital heart disease. It involves multiple heart defects that affect blood flow. Babies born with the condition may show signs of congestive heart failure. [my.clevelandclinic.org]

  Complications include elevated blood pressure, abnormal heartbeats, and heart failure. The treatment includes surgery to remove the lesions that cause blockages in the blood flow. [myacare.com]

  […] of heart failure, along with reduced cardiac output. [rcvmonline.com]

• Heart Murmur

  Some patients are diagnosed before birth, while others are diagnosed as a result of heart murmurs or other findings including: Poor weight gain. Rapid breathing. Shortness of breath (dyspnea), especially around feeding time. [my.clevelandclinic.org]

• Mitral Valve Prolapse

  Two years ago, he was operated on in another hospital based on the wrong diagnosis of mitral valve prolapse and subaortic membrane. [archivestsc.com]

• Prolapse

  Two years ago, he was operated on in another hospital based on the wrong diagnosis of mitral valve prolapse and subaortic membrane. [archivestsc.com]

• Suggestibility

  Another operation was suggested, but the patient refused. During the two year follow-up period, in spite of an atrial fibrillation attack and deteriorating dyspnea, the patient still did not want another operation. [archivestsc.com]

  The second 10/16 mm suggest embryonic residue with a dilated coronary sinus. (Fig. 2B). Undilated right ventricle with, good systolic function, No sign of pulmonary hypertension. [ncbi.nlm.nih.gov]

  We also found an abnormal vessel like density in 5-chamber view that suggested bleb sign and retroaortic course of the left circumflex (LCX). [rcvmonline.com]

Shone’s Syndrome Treatment: Things to Know Surgery is the ultimate treatment for Shone’s syndrome. A patient may need more than one surgery depending on the severity of the lesions. [myacare.com]

Earlier treatment may help save the aortic valve and thus avoid valve replacement. The treatment of choice is surgery. It cannot be repaired in the cath lab. In children, the membrane often regrows, sometimes rapidly. This is less common in adults. [achaheart.org]

Overview Symptoms and Causes Diagnosis and Tests Management and Treatment Prevention Outlook / Prognosis Living With Shone's Complex Overview Symptoms and Causes Diagnosis and Tests Management and Treatment Prevention Outlook / Prognosis Living With Back [my.clevelandclinic.org]

Conclusion Treatment required the coordinated efforts of a team of specialists. It could be either surgical with different method or by Trans catheter treatments. [ncbi.nlm.nih.gov]

Treatment and prognosis Surgical intervention is required in all cases. Generally, patients will need multiple surgeries at initial presentation, as well as repeated surgeries throughout their life 2,3. [radiopaedia.org]

Overview Symptoms and Causes Diagnosis and Tests Management and Treatment Prevention Outlook / Prognosis Living With Shone's Complex Overview Symptoms and Causes Diagnosis and Tests Management and Treatment Prevention Outlook / Prognosis Living With Back [my.clevelandclinic.org]

Treatment and prognosis Surgical intervention is required in all cases. Generally, patients will need multiple surgeries at initial presentation, as well as repeated surgeries throughout their life 2,3. [radiopaedia.org]

The severity of mitral valve obstruction still the main predictor of prognosis [1,2], the long-term prognosis with this complex is poor, with a mortality rate of 24%–27% [4]. [ncbi.nlm.nih.gov]

Since there is obstruction of flow into and out of the left ventricle, the prognosis depends on the degree of obstruction and its effect on blood flow. [en.wikipedia.org]

The prevalence of this syndrome is 0.6% of all cardiac malformations with a male: female ratio of 1.5:1.[6],[9] Patients with Shone syndrome have a poor long-term prognosis and often require multiple interventions in the early months of life. [rcvmonline.com]

Clinical discussion In this mini-review, we aim to describe this rare pathological condition its pathophysiological thoughts, and the way to diagnosis this complex early. [ncbi.nlm.nih.gov]

Prevention How can I prevent Shone’s complex? There is no guaranteed way to prevent having a child with Shone’s complex. [my.clevelandclinic.org]

In children with severe obstruction, the membrane may prevent enough blood from getting into the left side of the heart. This can lead to fluid backing up in the lungs. This is known as pulmonary edema. [achaheart.org]